The recurrent spontaneous formation of abscesses affecting multiple noncarious primary as well as permanent teeth is the principle clinical dental feature in cases of hypophosphatemia, a condition inherited through the X chromosome. Patients often have high pulp horns, large pulp chambers and dentinal clefts. We report a case of hypophosphatemic vitamin D-resistant rickets in a patient who reported to our department on multiple occasions with spontaneous abscesses in relation to his primary teeth. The aim of this article is to review the features of this disorder and to discuss the risks and benefits of the treatment options suggested in the literature.