[Angiomyolipoma and polycystic kidney secondary to Bourneville's tuberous sclerosis]

We describe the case of a previously healthy young woman admitted to hospital for abdominal pain and symptoms of shock. The preoperative diagnosis arrived at was polycystic kidney disease associated with ruptured angiomyolipoma. This combined pathology is pathognomonic of tuberous sclerosis; skin signs of the disease were also present in the patient, although undetected till then. The lack of neurological symptoms was an extraordinary finding. Cure is achieved at the cost of nephrectomy.