Hypophosphatasia is a rare inherited metabolic disorder characterized by a deficiency of alkaline phosphatase activity. It is characterized by a defect in bone and tooth mineralization. There are remarkable dental manifestations, mainly, the premature loss of fully-rooted deciduous teeth and delayed eruption of permanent teeth. This clinical report describes the oral management of a patient with juvenile hypophosphatasia, followed 10 years in the department of dentistry at the Rabta Hospital-Tunis-Tunisia. Removable prostheses were made to establish an acceptable masticatory function, speech, occlusion and aesthetics for the patient.