Cerebellar abiotrophies have a non-sex-linked, autosomal, recessively inherited basis in anumber of animal species, and lesions typically reflect progressive loss of Purkinje cells. In this report, ataxia was observed in a one-week-old male Samoyed puppy with no history of trauma or any other illness. Physical examination revealed uncoordinated movements, mild trunkal ataxia and intensive head tremors, so the dog had trouble eating, evacuating and difficulties in standing and walking without falling. The complete cell count, cerebrospinal fluid analysis and cerebral radiographs were normal. Treatment with diazepam, vitamin B1 and glucocorticoids had no effect on clinical signs and the dog died after a month. According to clinical signs and histopathological features, cerebellar cortical abiotrophy was diagnosed. This is the first report of a cerebellar cortical abiotrophy in a Samoyed puppy.