Hypertension in children with neurocutaneous disorders (phacomatoses) is a well-recognized complication of these diseases and the cause of hypertension is fairly specific within each group. In patients with neurofibromatosis, hypertension is mainly caused by renovascular disease, whereas in tuberous sclerosis (TSC) reasons for hypertension are renoparenchymal lesions, such as angiomyolipoma or cysts. We report on a girl with TSC and hypertension due to unilateral renal artery stenosis associated with aneurysmatic changes of internal carotid artery. This unusual combination of symptoms in our patient supports the importance of thorough and complete investigation of hypertension in children with phacomatoses.