Polycystic kidney disease with tuberous sclerosis (PKD-TSC) is characterized by early-onset and severe polycystic kidney disease with various manifestations of tuberous sclerosis (multiple angiomyolipomas, lymphangioleiomyomatosis and periventricular calcifications of the central nervous system). The disease is MONDO_0010856 (autosomal dominant polycystic kidney disease type 1 with tuberous sclerosis). Also known as: tuberous sclerosis/polycystic kidney disease contiguous gene syndrome.