A 45-year-old lady with good performance status (PS) and otherwise unremarkable personal and family history presented in February 2019 with lower abdominal pain and dysuria for 5 months, having failed conservative measures. Ultrasound abdomen and cystoscopy revealed a left lateral bladder wall (3 × 3 cm) submucosal growth. Transurethral resection biopsy specimen showed intact transitional lining epithelium with an invasive lobular tumor in subepithelium arranged in the form of interlacing fascicles and storiform pattern comprising of eosinophilic spindle shaped tumor cells with cigar-shaped nuclei, perinuclear vacuoles and moderate amount of eosinophilic cytoplasm. The tumor cells showed brisk mitotic activity, cytological atypia and large areas of necrosis. Immunohistochemistry (IHC) was positive for smooth muscle actin (SMA) and negative for GATA3 and cytokeratin. A diagnosis of leiomyosarcoma was given. Whole body 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)-contrast-enhanced computed tomography (CECT) showed an exophytic heterogeneously enhancing soft tissue mass with central necrosis (4.9 × 5.3 × 4.6 cm, SUVmax 15.3) arising from left lateral bladder wall and having ill-defined planes with uterus at places, multiple pelvic (left external iliac, internal iliac and common iliac, largest 1.3 × 1.1 cm, SUVmax 8.7) and paraaortic nodes (largest 0.8 cm, SUVmax 7.6) and a subcm-enhancing nodule in body of right adrenal with doubtful significance (SUVmax 4.8) (Fig. 1A, B). With a diagnosis of stage IV leiomyosarcoma of bladder, she received palliative chemotherapy with VAC/IE regime (vincristine 1.4 mg/m2 + adriamycin 75 mg/m2 + cyclophosphamide 1200 mg/m2 iv day 1, alternating with ifosfamide 1.8 g/m2 + etoposide 100 mg/m2 day 1–3, every 3 weeks). After 3 cycles, she had persistent symptoms and local progression on CECT, for which she received palliative radiotherapy to pelvis, 30 Gy in 10 fractions, and second line chemotherapy (gemcitabine 1000 mg/m2 days 1 and 8 + docetaxel 75 mg/m2 day 1 every 3 weeks) for 6 cycles. She had recurrent hematuria, and imaging suggested progressive bladder growth and unchanged nodal disease (Fig. 1C, D). Following multidisciplinary clinic discussion, she was advised surgery (radical cystectomy, total abdominal hysterectomy, pelvic lymphadenectomy, paraaortic sampling) in view of good PS and persistent symptoms. Intraoperatively, a large fungating solid growth (9 × 6 cm) arising from left lateral bladder wall, and adherent to left lateral pelvic wall and an ileal loop was seen. Bilateral ureters, uterus and vaginal cuff were uninvolved. There were no peritoneal deposits or ascites. Left paraaortic and aortocaval nodes were enlarged and firm in consistency. Bilateral pelvic nodes were not enlarged. Postoperative histopathology showed a polypoidal mass of 6.5 × 6 × 5 cm with perivesical fat invasion and close circumferential margin (1 mm). Morphology showed a spindle cell tumor cells arranged in short and long fascicles, with high mitotic activity. Morphology and IHC were identical with initial biopsy. Overall picture confirmed the earlier diagnosis of pleomorphic leiomyosarcoma (Fig. 2A–D). Pelvic and paraaortic nodes showed granulomas secondary to tumor antigen. Ziehl–Neelsen staining for acid fast bacilli was negative in the lymph nodes. The initial diagnosis of metastatic disease (in paraaortic nodes) was revised since the nodes had remained unchanged during serial imaging, and she was offered adjuvant radiotherapy (50.4 Gy in 28 fractions) based on NRG guidelines, after due consideration of prior radiotherapy dose and volume [3] (Fig. 3). Two months after radiotherapy, she reported to emergency with projectile vomiting, constipation and upper gastrointestinal bleed. Acute intestinal obstruction related to treatment-related pelvic fibrosis was suspected. CECT abdomen showed jejunal and ileal dilatation with no definite growth or site of obstruction. Emergency laparotomy was done and an obstructing growth 5 × 4 cm arising from intestinal mucosa at ileal anastomotic site was seen on exploration. Resection and anastomosis were done. Histopathology of the intestinal mass showed an invasive well-demarcated tumor apparently arising from muscularis mucosae and extending transmurally to reach the serosa. There was brisk mitotic activity and microscopic foci of necrosis. No lymphovascular or perineural invasion were identified. Resection margins and mesenteric margins are free. Four lymph nodes were identified that showed reactive changes. IHC was positive for SMA and negative for CD34, DOG1 and c-kit, ruling out the possibility of gastrointestinal stromal tumor. The histopathologic picture suggested metastatic leiomyosarcoma (Fig. 2E–H). Within 6 weeks following surgery, she again presented to emergency with a progressive intraabdominal (left lumbar) soft tissue swelling, initially suspected as an abscess based on ultrasound, but subsequently proven metastatic on cytology (Fig. 4). PET-CECT showed multiple paraaortic nodes (largest 1.6 cm, SUVmax 5.7) and serosal deposits (largest 7.3 × 4.6 cm, SUVmax 13.8) in pelvis with central necrosis and ill-defined fat planes with sigmoid colon, ileal loops, and left external iliac vessels (Fig. 5). Due to low PS, she was advised metronomic chemotherapy [4]. However, she could not start the same due to declining oral intake and succumbed to her illness 2 months after last PET imaging, with an overall survival of 1.7 years since diagnosis.