Background : Hypophosphatemic Rickets (HR) is a disease that affects mineralized structures including bone and dentine, studies on dental development in these patients are scarce with equivocal results. Aim : To assess dental development of a group of children with (HR) and to compare that to healthy matched controls, and to assess relationship between delayed medical treatment and dental development. Materials and methods : This is a controlled cross-sectional study carried out on a sample of 21 children with HR and healthy age and sex matched controls, diseased children were diagnosed at different ages. Dental age was assessed using Demirjian et al method. The difference between ages of study and control groups was assessed using t-test, Pearson correlation was used to test relationship between age of commencement of treatment and dental development delay. Results : Most HR subjects demonstrated dental delay ranging from 0.2 to 2.5 years which was significant (p-value = 0.028). The difference between dental age of the study and control groups was statistically significant using paired t-test. There was no correlation between age of commencement of treatment and amount of dental delay. Conclusion : Dental development was significantly delayed in a group of HR patients compared to matched healthy controls. Delay in commencement of treatment may lead to a permanent deficit in dental development.