Abstract
A radical nephrectomy specimen was referred for pathological examination. The history, course and the pathological findings proved very interesting. The specimen belonged to a forty four year old HIV positive man who was admitted with pain and tenderness in the right hypochondriac region. Sonography demonstrated fatty liver and incidental multilocular enhancing cystic lesion in the left kidney. A left radical nephrectomy was performed and pathological examination of the specimen showed a multilocular cystic renal cell carcinoma (MCRCC). The clinical presentation, management and outcome of this new entity are discussed.