Hanhart's syndrome, an uncommon disorder characterized by severe micrognathia, hypoglossia and absence of the extremities, belongs to the oromandibular-limb hypogenesis group of diseases. Our patient was admitted with the complaints of abnormality of the mouth and absence of the upper extremities. Physical examination revealed severe micrognathia, a small tongue adhered to the base of the mouth, absence of bone, muscle and skin tissue at the lower edge of the right humerus and at the middle zone of the left ulna and radius. According to these findings, the diagnosis of hypoglossia-hypodactylia (Hanhart's) syndrome was made. At the age of 1 1/2 years, tympanometric examination revealed conductive hearing loss due to dysfunction of the eustachian tube. Auditory brainstem responses also revealed bilateral severe sensorineural hearing loss. A literature search revealed two cases of Hanhart's syndrome with conductive hearing loss, but no report of sensorineural hearing loss as part of this entity. Therefore, we present this case of Hanhart's syndrome with severe sensorineural hearing loss.