Congenital portosystemic shunts (CPSS) were diagnosed in 46 dogs. The historic, physical, and laboratory findings were tabulated. Half of the affected males were cryptorchid. Urolithiasis was detected in 20% of the dogs. The biochemical tests with the best sensitivity for the diagnosis of CPSS were sulfobromophthalein retention, fasting serum ammonia concentration, and serum alkaline phosphatase activity. The survival time and quality of life were assessed by physical and biochemical reevaluation of the dogs and by means of a questionnaire that was completed by the owners. Five dogs were treated medically. Thirty-three dogs were treated surgically. Dogs that had complete surgical occlusion of the CPSS became normal, and quality of life was excellent. Dogs that had partial occlusion of the CPSS improved, and some became clinically normal. Dogs that did not have surgical correction of the CPSS had continuation of signs, but several survived for years.