Duane retraction syndrome is an ocular motility disorder with which an increasing number of congenital abnormalities appear to be associated. In the present paper the authors report a case of bilateral Duane retraction syndrome with an extraordinary hand abnormality. Numerous theories concerning the etiology and pathogenesis of Duane retraction syndrome and its relationship to the associated findings have been proposed, but the exact mechanism is still under investigation. Further multi-centered studies and the classification of the associated clinical findings in an organized manner may help to explain this mechanism.