Chronic renal failure as consequence of renal dysplasia was diagnosed in three young adult Dutch kooiker dogs (Dutch decoy dogs). Two animals were anorectic from an early age and were thinner than healthy dogs of the same breed. All three were presented because of apathy and weakness. Laboratory examination revealed anaemia and uraemia. One dog was presented with severe dehydration and died during emergency treatment. One dog was euthanatised because of a poor prognosis, and one was given a low‐protein diet. This dog survived for 7 months after the diagnosis of chronic renal failure. At necropsy all three animals had shrunken, pale, and firm kidneys that showed microscopical lesions characteristic of canine renal dysplasia, such as asynchronous differentiation of nephrons, persistent immature mesenchyme, persistent metanephric ducts, and adenomatoid proliferation of the tubular epithelium. Secondary degenerative and inflammatory changes consisted of interstitial fibrosis and predominantly lymphocytic/plasmacytic inflammation. This is the first report of renal dysplasia in the Dutch kooiker dog. The disease should be included in the differential diagnosis in young Dutch kooiker dogs with signs of chronic renal failure. The presentation of three cases of this rare disease in this breed, which is based on a rather small gene pool, suggests that it is a familial or hereditary nephropathy.