A case of cervical canal stenosis accompanied by congenital cervical fusion and extracerebral occlusive disease of the verebral artery was reported. A 39-year-old male was admitted to our hospital on June 5, 1976, because of his shoulder strain, motor weakness and numbness of his left hand, and gait disturbance for a couple of months. Neurological examination disclosed spastic paraparesis with positive pyramidal signs in the four extremities which was greater on the left side, and the superficial and deep sensations were diminished below C-4 dermatomes bilaterally. Spinal tap revealed crystal clear fluid under an initial pressure equivalent to 120 mm of water with a protein content of 20 mg/dl. Plain X-P and laminogram of the cervical spine showed congenital fusion of C 2-3, cervical canal stenosis and cervical spondylosis. Posterior spurring of C 3-4 was demonstrated only on the laminogram. Myelogram showed incomplete myelographic block at the site of C 3-4-5. The left retrograde brachial angiogram disclosed occlusion of the vertebral artery at the site of C3-4. where the proxymal vertebral artery anastomosed with the well developed muscular branch of the vertebral artery, taking a tortuous course at the leve of the transverse foramen of the atlas, and were opacified the distal vertebral artery and basilar artery. Each collateral flow extending from the deep cervical artery and ascending cervical artery anastomosed with the proxymal and distal portion of the occluded vertebral artery. In spite of conservative treatment for two weeks, the cord compression syndromes rather deteriorated. Posterior decompression, that is, C 2-7 laminectomy and C 3-4 partial facetectomy were performed for relieving the symptoms. The postoperative course was uneventfull and there was progressive improvement of the sensory, motor and gait disturbance. The relationship betwen the cervical canal stenosis and cervical spondylosis and their clinco-radiological problem were discussed. The close embryological relationship was very suggestive of the pathogenesis between the cervical bony lesion and congenital cervical fusion and the occlusive vascular lesion of the vertebral artery.