The most common site of origin is in the abdominopelvic region,gastrointestinal tract, retroperitoneum, and uterus.1,2 Most PEComas are often related to thetuberous sclerosis complex (TSC).1 They are mostly benign and curable by surgical resection when benign.2 Here, we describe a case of a young male who had progressive chronic hip pain thatwas found to have a PEComa associated with TFE3 translocation in his pelvicregion. This evidence concerns the gene TFE3 and neoplasm with perivascular epithelioid cell differentiation.