Diffuse cystic renal dysplasia and hepatic fibrosis is an inherited developmental disease of dogs. Affected dogs are born with abnormalities or complete absence of hair-like structures called cilia which play an important role in organ development and other developmental processes. Lack of functional cilia in affected dogs results in severe malformation of the kidneys and scarring (fibrosis) of the liver during fetal development. Kidneys of affected dogs are typically enlarged due to formation of large cysts throughout the tissue. In addition, affected dogs are often born with poorly developed lungs and may also display a cleft palate, free fluid in the chest or abdominal cavity, or malformations of the diaphragm. Affected puppies typically die within a few days of birth.