During the last 21 years seven adult captive Brazilian agoutis (Dasyprocta leporina) from four different zoological gardens were necropsied and histologically examined at the Leibniz Institute for Zoo and Wildlife Research, Berlin, Germany. All animals had polycystic kidney disease as the major pathological change. Except in one case, no clinical signs were recognized prior to death. The animals had macroscopic bilateral alterations of the kidneys ranging from granulated surfaces to severe polycystic changes. Microscopic examination revealed multifocal to generalized, moderate to severe cystic dilatations of Bowman’s capsules and renal tubules, moderate mesangial and capsular proliferation of the renal corpuscles, mild interstitial fibrosis and mild to moderate interstitial lympho-plasmacytic infiltrations. Little information is known about the genetic relationships of these animals, but breeding practice indicates a high possibility of inbred agouti zoo populations in Germany. This is the first report on polycystic kidney disease in Brazilian agoutis with presumed genetic background.