Abstract
PEComas or Perivascular Epithelioid Cell neoplasms are uncommon mesenchymal origin neoplasms composed of perivascular epithelioid cells. They affect various anatomic sites, kidneys being one of them. Renal PEComas are further rare, posing a challenge to include them in the differential diagnoses. We present a case of a type of renal PEComa in a 54-year old post-menopausal female with vague abdominal pain. Imaging revealed a large left perinephric mass initially suspected to be a liposarcoma. Surgical excision and subsequent histopathological evaluation confirmed a benign, fat predominant renal angiomyolipoma. This case depicts the mimicking and heterogenous nature of PEComas and the importance of early detection followed by appropriate treatment.