Renal cystic disease compromises a diverse group of inherited and acquired entities. This article reviews the clinical, pathologic, and radiologic findings of eight renal cystic diseases. For each entity, the current concepts of pathogenesis and pathophysiology are discussed. When appropriate, controversies concerning terminology, management, and malignant potentials are addressed. Renal cystic diseases that are discussed include autosomal dominant and autosomal recessive polycystic kidney disease, medullary sponge kidney, medullary cystic disease, multicystic, dysplastic kidney, von Hippel-Lindau disease, acquired cystic kidney disease, and tuberous sclerosis.