A case of hereditary multiple exostoses with acute cervical myelopathy, tetraplegia, and apnea is reported. Neurological complications as a result of osteochondromas in hereditary multiple exostoses are rare. The majority of osteochondromas in the cervical spine arise from the neural arch. Magnetic resonance imaging and computed tomography are invaluable in localizing the origin of the lesion and its relationship to the spinal cord. Decompressive laminectomy usually results in excellent functional recovery. Where signifciant dorsal spinal cord compression exists without neurological deficit, prophylactic decompression can be recommended.