A 4-year-old female French bulldog presented with a 6-month history of right-sided head tilt and acute onset ataxia. Magnetic resonance imaging (MRI) showed a large mass lesion at the cerebellomedullary pontine angle. The dog was able to stand and walk after beginning symptomatic therapy with prednisolone, acetazolamide and glycerin. Magnetic resonance imaging 10 months after the first examination indicated slight expansion of the tumor. The dog was able to walk with continuous symptomatic therapy for 15 months until death, although the head tilt persisted. On postmortem examination, the gross tumor was slightly larger than when seen on the second MRI scan and was histopathologically diagnosed as a choroid plexus papilloma.