The Fraser syndrome is an autosomal recessive disorder, that doesn't have an exact pathogenesis known, yet. Up to the date, 117 cases were reported, in the medical literature. All cases reported present craniofacial disorders, 93% with cryptophthalmos and 44% with otological disorders, one third of them, with atresia of the external auditory meatus. There is no report, in the medical literature, of the use of BAHA® system (Bone Anchored Hearing Aid) for the treatment of hearing loss, in these patients. We present one case report of the use of BAHA® system for the treatment of hearing loss, in a patient with Fraser syndrome, with bilateral moderate to severe mixed hearing loss.