Multicystic kidney disease (MCKD) is the most common form of Congenital Abnormality of Kidney and Urinary Tract (CAKUT). This anomaly of renal development is characterized by unilateral enlarged cystic formations and fibrous dysplastic parenchyma. The long-term prognosis is usually good; however because of reduced nephron mass, an early prevention of cardiovascular risk and nephrotoxicity is recommended. A lifelong follow-up of blood pressure, serum creatinine and microalbuminuria seems logical as well as in other patients with a single kidney. MCKD is usually diagnosed during pregnancy so that parents often question about long-term prognosis and follow-up. Therefore, we propose an information sheet for parents.