Acrodysostosis is a very rare congenital disorder. Less than 80 cases have been reported in the medical literature until now. Most of the reported cases were sporadic, but in some families evidences like father's old age and affected parent and child gives rise to autosomal dominant inheritance. clinical symptoms are obvious enough and experienced teachers can easily diagnose the disease. In this care report, a 21-year-old Iranian male patient, who showed completely the symptoms of acrodysostosis, is reported. According to our experience, this is the first known case in Iran. There must be other cases in mentally retarded patients who are dysmorphic. Therefore, it is necessary to be aware of symptoms of this disease when facing with dysmorphic mentally retarded patients.