Cystic kidney disease (CKD) encompasses a wide spectrum of pathologies caused by different variables that disturb the nephrogenesis and renal maturation process. CKD can be divided into hereditary and non-hereditary disease. Advances in genetics have led to changes in the way we classify and define each of these diseases. Cysts are a result of disturbances between the epithelium and the stroma as a consequence of having obstructive effects for long periods of time due to degenerative disorders or neoplastic and mutagenic mechanisms. The objective of this review is to define CKD in a broad and comprehensive way. We performed a systematic review of the literature in mEdlinE for publications that included the terms dysplastic kidneys, multicystic dysplasia and kidney dysplasia. We reviewed various important publications which we selected, analyzed and referenced in this article.