Authors present the review of current literature on the subject of nephrological complications in tuberous sclerosis complex (TSC) on the basis of two ease reports of children with familial TSC. In 18-years-old girl the features of polycystic kidney disease with end-stage renal failure, epilepsy, mental retardation, calcifications of structures in central nervous system and skin abnormalities coexist. In 10.5-years-old boy morphological changes in both kidneys revealed hyperechoic renal cortex (renal function - creatinine clearance within normal ranges), other features are similar as compared to sister. In the girl during the one month of observation asymptomatic acute bleeding to a cyst and under the renal capsule occurred. It was necessary to perform bilateral nephrectomy because of these life threatening events.