Abstract
PEComas or perivascular epithelioid cell tumors are rare soft-tissue tumors that form around perivascular spaces with a female preponderance, the origins of which are yet to be established. PEComas of the gastrointestinal tract are very rare, and only 21 cases have been reported in the literature. This case report comes from a tertiary hospital of an adolescent male who presented with complaints of vague abdominal pain and was noted to have lump in his right hypochondrium. Ultrasound-guided biopsy was done, after which PEComa was considered and was further confirmed on the excised specimen using HMB-45, the characteristic melanin marker of PEComa on immunohistochemistry. The adolescent is now healthy and on regular follow-up. PEComas are rarely considered in the first few differential diagnoses, as they require histopathological confirmation for definitive diagnosis.