A 15 wk old male unilateral cryptorchid German shepherd dog weighing 18 kg was referred for a nonhealing cutaneous lesion dorsally at the level of the sacrum, urinary incontinence, and a deviated tail. MRI revealed spina bifida and meningomyelocele continuous with the skin surface. Surgical correction of the meningomyelocele involved closure of the open meningeal defect, transection of the abnormal spinal nerves to the skin surface, and closure of the skin defect with a good outcome. Histopathology confirmed a meningomyelocele. This case report describes the MRI findings, surgical procedure, and proposed pathogenesis of spina bifida and meningomyelocele in a dog.