The Oto-palato-digital syndrome is a generalized skeletal dysplasia resulting in short stature, characteristic facies, and unusual deformities of the hands and feet. In addition, cleft palate and other congenital malformations are present. Some patients have conductive deafness due to dysplastic auditory ossicle which is sometimes stapes fixation. We experienced a case of Oto-palato-digital syndrome which showed bilateral stapes fixation, high-arched palate and syndactly of toes. Congenital stapes fixation was treated through partial stapedectomy with good hearing gain.