Cervical spondylotic amyotrophy is characterized with weakness and wasting of upper limb muscles without sensory or lower limb involvement. Two different mechanisms have been proposed in the pathophysiology of cervical spondylotic amyotrophy. One is selective damage to the ventral root or the anterior horn, and the other is vascular insufficiency to the anterior horn cell. Cervical spondylotic amyotrophy is classified according to the most predominantly affected muscle groups as either proximal-type (scapular, deltoid, and biceps) or distal-type (triceps, forearm, and hand). Although cervical spondylotic amyotrophy always follows a self-limited course, it remains a great challenge for spine surgeons. Treatment of cervical spondylotic amyotrophy includes conservative and operative management. The methods of operative management for cervical spondylotic amyotrophy are still controversial. Anterior decompression and fusion or laminoplasty with or without foraminotomy is undertaken. Surgical outcomes of distal-type patients are inferior to those of proximal-type patients.