This is a case report of a patient with angiomyolipomas in both kidneys which was confirmed in one kidney by open biopsy and histologic examination. Two years after diagnosis of the angiomyolipomatosis, the patient died of metastatic hypernephromas of both kidneys. The tumors had affected the kidneys almost totally; only in the right kidney were remnants of the angiomyolipoma still recognizable. A review of the literature shows how rare is the simultaneous occurrence of angiomyolipomas and hypernephroma.