1/3 to 1/2 of PEComa is clinically associated with tuberous sclerosis complex (TSC) (11) with germline mutations in TSC1 (9q3.4) and TSC2 (16p13.3) genes, this case did not detect germline mutations in the TSC gene and is consistent with a case of sporadic PEComa. Here, TSC1 is linked to neoplasm with perivascular epithelioid cell differentiation.