A case of Klippel-Feil syndrome with crossed renal ectopia with fusion and unilateral vertebral artery occlusion is reported. A 61-year-old female was admitted because of having developed myelopathy, gait and sensory disturbance. The physical examination on admission revealed spastic gait and hypesthesia of the lower extremities. A hand size, elastic hard tumor mass was palpable at the right lower abdomen. The cervical radiogram showed fused 5th, 6th and 7th cervical vertebrae and severe spondylotic changes. Laminectomy was performed because of severe compressions of the cervical cord, evaluated by Metrizamide CT scans and MRI. The left subclavian angiogram showed occlusion of the left vertebral artery, and excretory pyelogram and abdominal CT scans showed crossed renal ectopia with fusion. 99mTc-DTPA renoscintigram revealed poor RI uptake and low glomerular filtering rate of ectopic kidney. The patients with Klippel-Feil syndrome are at greater risk of having a renal anomaly and a vascular accident of the vertebral artery. Examinations of urogenitary organs and vertebral arteries are necessary for the treatment of this syndrome.