Skin changes associated with carpal tunnel syndrome have rarely been reported. A 60-year-old patient was referred to our Department of Dermatology for evaluation of an aseptic gangrene of the distal phalanx of the third finger of her left hand, progressive nail dystrophy, sclerodactylia and spontaneous ulcerations. These features were seen on the digits with sensory supply from the median nerve. The patient had hand paresthesias for at least 10 years. No abnormal results were found in biochemical, hematological or immunological determinations. An echo-Doppler study of the upper limbs was normal. An electromyographic study was consistent with a bilateral carpal tunnel syndrome (CTS). Ulcerative and mutilating CTS can occasionally produce sclerodactylia with ulcerative lesions on distal fingers, nail dystrophy and acro-osteolysis as a consequence of mechanical compression of the autonomic fibers of the median nerve. The clinical picture includes Raynaud's phenomenon in some patients. Correct diagnosis is important, since ulcerative and mutilating CTS is a marker of a severe neural lesion, which is amenable to treatment by surgical decompression.