There are reports in the literature of the various dental features of hypophosphatasia, especially where it affects the deciduous dentition. The descriptions include both the manifestations of the disorder and the subsequent patterns of tooth loss. There are fewer descriptions of the effects of hypophosphatasia on the permanent dentition and little information on the subsequent prosthodontic management of these patients, particularly in relation to the use of dental implants. The aim of this paper was to review the literature on the dental effects of hypophosphatasia, present two cases and describe how one of those patients, a young adult, was successfully rehabilitated using dental implants. That latter patient's pattern of tooth loss as well as some histological and scanning electron microscopic findings of root cementum from the other case is also described.