We report on several relatives in 5 generations of one family with prominence of the ears, with a marked constriction at the junction between the lower and middle thirds of the pinna. Computerized tomography and radiographs in the propositus and his affected father showed abnormalities of the condyle of the mandible. The propositus had more severe changes in the condyle with microstomia and reduced range of motion of the mandible in the temporomandibular joint. There was no hearing loss or abnormalities of the bones of the middle ear.