Reports of a mild form of hypophosphatasia have been presented in which the oral findings were the only clinical and radiographic manifestations of the disease. It is important to support the clinical interpretations with laboratory findings in order to make a differential diagnosis from such diseases as Papillon-LeFevre Syndrome and cyclic diseases and noncyclic neutropenia. The term odontohypophosphatasia is suggested in those cases where the dental findings are the predominant manifestations of the disease.