During March 1988, a case of bilateral polycystic kidney disease (PKD) occurred in an aged raccoon (Procyon lotor) at a zoo in Wilmington, Delaware (USA). Prior to its death, the raccoon had no clinical signs. On necropsy there was bilateral enlargement of kidneys which, on cut sections, had many variable sized fluid-filled cystic cavities. Endometrial hyperplasia with presence of multiple variable-sized cysts were also seen in the uterus of this raccoon. Microscopical examinations were characteristic of an end-stage renal failure due to PKD. Neither PKD nor cystic endometerial hyperplasia appears to have been previously described in this species.