Variations in the medial triceps in conjunction with bilateral ulnar neuropathy have been identified in three generations of one family also possessing the phenotype of Waardenburg syndrome (a rare autosomal-dominant disorder with clinical features including cochlear deafness, dystopia canthorum, and pigmentation problems). To our knowledge, no other inherited condition with triceps anomalies has been reported. Study of this family provided insight into the relationship between dislocating medial triceps and ulnar neuropathy and demonstrated that a broad spectrum of clinical presentations exists-from being completely asymptomatic to producing symptomatic snapping and ulnar neuropathy.