Oral-facial-digital syndrome is a group of congenital anomalies, which affects the face, oral structures and digits. There are nine subtypes. OFDS type I, is x-linked dominant trait mostly affecting females. Reports of OFDS type 1 in Asians are extremely rare. This paper shows a case of OFDS type 1, in a southern Chinese girl, who in addition to most of the classic features, had fusion of the mandibular canine and lateral incisor teeth.