An 8-month-old female Saint Bernard dog was presented with gait abnormalities consistent with a left-lateralizing cervical myelopathy. Imaging revealed a large, irregular soft tissue and mineral mass at the level of C1 and C2. The lesion was successfully excised, and histopathology was performed, revealing evidence of both multiple cartilaginous exostoses and calcinosis circumscripta. To the authors’ knowledge, this is the first report comparing features using magnetic resonance imaging, computed tomography, and radiography. Additionally, multiple cartilaginous exostoses have not previously been reported to occur in combination with calcinosis circumscripta.