This paper reviews the literature on prepubertal periodontitis (PP) according to the definition and the classification of PP by Page. The generalized form of PP (G-PP) defined by Page was identified as the oral manifestation of a systemic disease called leukocyte adhesion deficiency (LAD) and thus the pathogenesis is known at the molecular level. On the other hand, the localized form of PP (L-PP) is a disease defined by clinical criteria and its pathogenesis is not known at the molecular level. The reported prevalence of L-PP differs widely in reports, according to the methodology and population studied. However, it seems to be at least 0.84% or greater. Potential periodontal pathogens that have been associated with L-PP include the following bacteria: Actinobacillus actinomycetemcomitans (A.a.), Bacteroides intermedius, Bacteroides gingivalis, Capnocytophaga sputigena, and Eikenella corrodens. Suggested contributing factors have included PMN or monocyte chemotactic defects and cementum defects. There is no unique pattern of bone loss in L-PP; however, there may be a type of periodontitis that affects all deciduous teeth, in the absence of LAD. L-PP may lead to localized juvenile periodontitis (LJP) or generalized juvenile periodontitis (GJP). Histiocytosis X, hypophosphatasia and mild forms of blood dyscrasias may have minimal or no clinical signs and symptoms except for alveolar bone loss. Therefore diseases known to be associated with alveolar bone loss must be definitively excluded in prepubertal children until the pathogenesis of L-PP at the cellular and molecular levels is understood and L-PP can be definitively diagnosed either as a distinct disease entity or entities.