Hoyeraal-Hreidarsson syndrome (HHS) is a severe multisystem disorder associated with premature mortality, due to bone marrow failure. Because HHS is a very rare disease and almost all cases die before 7 years of age, the dental development and dental findings of HHS patients are still not clear. According to our knowledge only twenty seven cases were reported up to now and we did not find any report focusing on the dental evaluation of HHS patients. Therefore, in this report, we aimed to present the dental findings of a twenty-year old patient with HHS.