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Background: Hypophosphatemic rickets (HPPR) and hypophosphatasia (HPP) are hereditary pathologies accompanied by phosphorus metabolism problems and rickets deformities of the skeleton. Periodontal diseases, their diagnostic methods and factors affecting the progress of periodontal tissue damage are the least studied among the dental manifestations of HPPR and HPP., Objective: The aim of present cohort study was evaluation of periodontal tissue status in adolescents with HPPR and HPP., Materials and Methods: A dental examination of 20 adolescents aged 12 to 18 years with genetically and biochemically confirmed hereditary rickets-like diseases (E83.3 Disorders of phosphorus metabolism and phosphatases: hypophosphatemic rickets (HPPR) and hypophosphatasia (HPP)) was performed at the Pediatric Dentistry Department of Moscow State University of Medicine and Dentistry. In the present study clinical examination and cone-beam computed tomography (CBCT) data of patients were analyzed., Results: According to the clinical examination data, a strong direct correlation was found between patient's age and the periodontal diseases severity (correlation coefficient r =0.87) in the study group. CBCT revealed the significant alveolar bone hypomineralization in adolescents with HPPR and HPP in comparison to control group ( p <0.05). According to the measurement results obtained a strong inverse correlation between alveolar bone mineral density and alveolar bone reduction (correlation coefficient r = -0.74) in adolescents with hereditary rickets-like diseases was revealed., Conclusion: The diagnostic options of CBCT allow to begin comprehensive dental treatment of patients with HPPR and HPP at an early stage of periodontal disease preventing further development of pathology.