Clear cell myomelanocytic tumours are extremely rare neoplastic growths considered to be members of the family of perivascular epithelioid cell tumours (PEComas), which have in common the coexpression of melanocytic and smooth muscle immunohistochemical markers. These tumours are known to be ubiquitous with uncertain tumour biology and to have unpredictable clinical behaviour. They have been reported in the genitourinary tract, including the kidney and prostate. There are only 3 reported cases of clear cell myomelanocytic tumours originating in the urinary bladder. We report a case of a 24-year-old woman with chronic pelvic pain who underwent laparoscopic partial cystectomy and total excision of a bladder mass. Pathological examination revealed primary PEComa of the urinary bladder. Subsequent follow-up procedures, including cystoscopy and imaging, have not revealed any evidence of disease recurrence. The patient remains clinically free of disease 3 months after surgery.
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