A 16 wk old intact female Gordon setter was examined for a 2 wk history of progressive cerebellovestibular ataxia. Eosinophilia was found on complete blood count, and the remaining blood work was normal. A trial treatment with clindamycin and anti-inflammatory prednisone did not result in improvement of clinical signs; therefore, the dog was euthanized. On histopathologic examination, cross sections of a 75 μm wide nematode larva with a 5 μm cuticle, prominent lateral cords, lateral alae, and coeloemyelian musculature were identified in a focally extensive region of cerebellar necrosis. The size and morphology of the parasites was most consistent with Baylisascaris procyonis. This case highlights a rare but important zoonotic disease that should be considered as a differential diagnosis in any dog exhibiting acute, progressive central nervous system signs, and peripheral eosinophilia. The index of suspicion should be elevated in dogs with an unknown deworming history and known or suspected exposure to raccoons. Because most anthelminthics have been shown to be effective against B. procyonis, annual deworming is recommended in the at-risk population to reduce the likelihood of aberrant migration and zoonosis.