Fifteen cases of renal angiomyolipoma not associated with tuberous sclerosis are presented. The clinical and radiological features, the pathology and the management of these rare but well-documented tumors are discussed. They pose problems in diagnosis, and controversy still exists regarding their management. Additional techniques such as ultrasonography and computerized tomography may help in the diagnosis. When conservative surgery is not possible, or when a solid tumor cannot be confidently excluded, nephrectomy should be performed.