Juvenile-type distal muscular atrophy of the upper extremities is a condition characterized by Juvenile onset, muscular atrophy limited to the hand and forearm, and lack of definite sensory disturbance. Diagnostic imaging techniques and operative findings showed compressive lesions of the cervical spinal cord in seven cases showing distal muscular atrophy of the upper extremities. In most of these cases, cord compression could be recognized, not during neck extension, but during flexion on myelogram, computed tomography, and magnetic resonance imaging. All cases underwent anterior spinal fusion to prevent flexion at the affected site, and the dural tube was opened to observe the surface of the cervical cord in four cases. It was confirmed that cervical myelopathy can occur even in young persons with no spondylotic change in the cervical spins.