A 37 years old woman presented with scleroderma en coup de sabre of the head with chronic homolateral ocular hypertony. Skin disorders appeared in the childhood and extended slowly until the age of 20. Glaucoma, found 5 years earlier, was unimproved by medical treatment; the opposite eye was normal. Two goniotomia in 1974 were uneffective. A trabeculectomy in 1975 was followed by scleral fistula with important ocular hypotonia. A scleral flap was followed by the disappearance of hypotonia with no return to previous hypertony. The connections between scleroderma and glaucoma are discussed; vascular and neurologic mechanisms are suggested. Up to now, glaucoma has been reported in patients with systemic soleroderma and Romberg disease.