Hallermann-Streiff syndrome is a rare congenital disorder characterized as a complex association of developmental anomalies involving structures of ectodermal origin (the face, the scull, the hair, the skin, the eyes, the teeth) and overall growth and development. Ophthalmic abnormalities included bilateral congenital cataract. Glaucoma is uncommon. We report a case with Hallermann-Streiffsyndrome having all the main features of the syndrome, however associated with juvenile glaucoma and without congenital cataract. An 16 year-old-boy was admitted in our hospital for surgical treatment of juvenile glaucoma. His ophthalmic features included microphthalmos, enophthalmos, short upper lid, and thin conjunctiva, edematous corneas with fine epithelial bulla, atrophic-colobomatous iris in the left eye, slightly dilated pupils and clear lenses. The right optic disc was pale and cupped (0.9-1). The left optic disc was pale. The IOP was 70 mmHg in the right eye and 62 mmHg in the left eye. There was no light perception on the right eye and 1/60 in the left improving to 0.1 with +7.0 Dsph. Antiglaucomatous therapy failed to reduce the pressure and a left trabeculecthomy was carried out. Protective eye shields during sleep were recommended to protect the corneas from dryness because of the short upper lids. In the left eye the IOP has fallen to within normal limits and the vision has improved 0.3-0.4 with +7.0 Dsph. The medical therapy was continued in the right eye but the pressure still remains high (40 mmHg).