Polycystic kidney and liver disease was seen in a stillborn white-tailed deer (Odocoileus virginianus) fawn. Bilaterally enlarged kidneys were characterized by severe dilatation of all renal tubules. Glomeruli were sparse, small, and located within a dilated Bowman's capsule. The liver was characterized by marked periportal fibrosis, biliary hyperplasia, and bile duct ectasia with dilated ducts containing inspissated bile. The presentation and morphology of this case are most similar to autosomal recessive polycystic disease in humans.