Renal cystic disease occurs as a result of cysts of varying sized localized in an enlarged kidney. It can be classified into different forms such as autosomal-dominant polycystic kidney disease, autosomal-recessivepolycystic kidney disease, unilateral renal cystic disease, renal simple cysts, multicystic dysplastic kidney,pluricystic kidney of the multiple malformation syndromes and medullary cystic disease. Recent studies showed that there is a relation between inherited cystic disease and changes in a group of genes involved in the formation and function of bothprimary cilia of the embryonic node and cilia in epithelial renaltubes. On the other hand, cysts in acquired conditions occur as a result ofobstructive, stromal–epithelial malinductiveand neoplastic mechanisms. Moreover, several genetic and acquired renal cystic diseases were found to be linked to the development of renal cellcarcinoma. There are different approaches to diagnose various types of renal cysts, while ultrasound and slice imaging are the main tools for the diagnosis of simple renal cysts, Bosniak classification was selected as the basic method for the diagnosis of renal cystic masses larger than 1 cm. In the current review we will discuss the various types of renal cystic diseases and the main diagnostic approaches.