An 11-year-old male Pekingese was evaluated because of a history of head tilt. Neurologic examination revealed a right-sided head tilt, ataxia, scoliosis, and proprioceptive deficits. Diagnostic testing included magnetic resonance imaging (MRI) of the head and neck. After IV administration of gadopentetate dimeglumine, an extra-axial, highly contrast-enhanced mass in the brainstem, cerebellar herniation, and syringohydromyelia were detected via MRI. The dog was treated with corticosteroids and radiation therapy of the mass for 4 weeks (total dose, 42.5 Gy). Magnetic resonance imaging was repeated 9 weeks and 6 months after radiation therapy; compared with the initial findings, a reduction in the size of the brainstem mass was observed in both MRI scans. The third MRI scan also revealed a normal cerebellar shape, no evidence of herniation, and resolution of syringohydromyelia in the dog at that time. It is recommended that whenever syringohydromyelia is observed via MRI, a primary cause (cranial or caudal to the affected region) should be sought.