INTRODUCTION:Hypophosphatemic rickets represents a group of heritable renal disorders of phosphate characterized by hypophosphatemia, normal or low serum 1,25 (OH)2 vitamin D and calcium levels. Hypophosphatemia is associated to interglobular dentine and an enlarged pulp chambers.AIM:Our goal was to verify the dental abnormalities and the oral health condition in these patients.MATERIAL AND METHODS:Prospective study of oral conditions in patients with Hypophosphatemic rickets. This report employed a simple method to be easily reproducible: oral clinical exam and radiographic evaluation.RESULTS:Fourteen patients were studied, 5 males, median age of 11years (4 to 26). Occlusion defects (85,7%) and enamel hypoplasia (57,1%) were significant more frequently than dental abscesses (one patient). We observed enlarged pulp chambers in 43% of the patients and hypoplasia and dentin abnormalities in 14,3%. We could not detect a significant correlation between dental abnormalities and delayed treatment (p>0,05). DMFT index for 6 to 12 years patients (n = 12) showed that the oral health is unsatisfactory (mean DMFT =  5).CONCLUSIONS:Patients with Hypophosphatemic Rickets frequently present dental alterations and these are not completely recovered with the treatment, unless dental abscess and they need a periodical oral examination.