Carpal tunnel syndrome (CTS) is characterized by burning pain, numbness and tingling sensation in the thumb, index and middle fingers and the lateral half of the palm and progressive atrophy of the thenar muscles by compression of the median nerve within the carpal tunnel due to a variety of etiologic factors. Surgical intervention usually successfully relieves symptoms of CTS. Recently CTS has been regarded as one of the major clinical manifestations of dialysis-related amyloidosis due to beta 2-microglobulin deposition and recognized with increasing frequency in patients undergoing long-term hemodialysis. We report a case of carpal tunnel syndrome due to dialysis-related amyloidosis in patients undergoing long-term hemodialysis, confirmed by electromyography and biopsy in transverse carpal ligament and median nerve.