Case report of a 52-year-old woman with angiomyolipoma of the left kidney. The tumor had appearance of a typical angiomyolipoma predominantly composed of spindle cells, some epithelio- id cells and few large hyalinized vessels. Adipose tissue was concentrated into small foci. Unusual presence of large cells with a clear fine granular cytoplasm closely resembled cells of the „sugar tumor“ of the lung. Epithelioid cells and occasionaly spindle cells were HMB45 positive. A minori- ty of cells also coexpressed S100 protein. Clear cells were usually strongly positive for HMB45 too. Our findings supported consideration of a close relation between clear cell („sugar“) tumor of the lung and angiomyolipoma.

        Key words: clear cell angiomyolipoma - kidney - clear cell tumor