Autosomal recessive polycystic kidney disease (ARPKD) is an inherited disorder characterised by the development of cysts affecting the collecting ducts. It is frequently associated with hepatic involvement. ARPKD is a rare disease that affects 1/40 000 children. Its prevalence in the general population is 1/85 000. After birth, in addition to nephromegaly, arterial hypertension and urinary tract infections are common and often severe. Liver involvement can be asymptomatic or may consist of portal hypertension or biliary duct infection such as cholangitis. Liver function remains normal. The disease follows a recessive pattern of transmission and the causative gene PKHD1, is located on the short arm of chromosome 6. It contains more than 80 exons and codes for a protein called fibrocystin or polyductin. Ultrasonography shows hyperechogenic and enlarged kidneys, with small cysts in some cases. Intravenous urography visualization of contrast medium in the collecting tubules is prolonged. On renal biopsy, cystic dilations of the collecting tubules following a radiate pattern are observed. Liver ultrasonography shows biliary duct ectasia, a heterogeneous liver and possibly signs of portal hypertension. Biopsy shows biliary dysgenesis with multiple and dilated biliary ducts and periportal fibrosis. Prenatal ultrasonography shows hyperechogenic, enlarged kidneys and, in the more severe forms, oligohydramnios. Renal failure is the major complication, but end-stage disease rarely occurs before the age of 15 years. Treatment for end-stage renal failure consists of dialysis and renal transplantation.