We report a case of unilateral renal angiomyolipoma in a 10-year-old boy wi
th tuberous sclerosis. The case is worthy of attention because the symptoms
occurred at an early age and because the lesion was unilateral, the latter
confirmed at 11-year follow-up. The presentation and treatment are discuss
ed, and the pertinent literature is reviewed. The case demonstrates that it
is of clinical importance to monitor renal involvement in children with tu
berous sclerosis. Copyright (C) 2001 S. Karger AG, Basel.