A case of Alport's syndrome and coincidental temporomandibular joint degenerative disease is reported. Alport's syndrome is an unusual genetic disease that ultimately results in renal failure and has a high incidence of sensorineural hearing loss. The patient presented had complaints of facial and joint pain that mimicked what is currently termed temporomandibular disorder, including headache, tinnitus, joint pain, and temporal swelling. The significance of renal osteodystrophy is briefly reviewed as it pertains to this clinical presentation. The clinician must be alert to the many potential causes of degeneration of the temporomandibular joint, one of which may be metabolic in origin.