The role of the long head of biceps brachii tendon in the stahilization of the head of the humerus is a highly interesting issue in recent studies. But, it is not well understood. Congenital absence of biceps long head tendon ot the hilateral shoulder joint is very rare and on review of literatures, we could not find any case reported. We experienced one case which was confirmed by magnetic resonance imaging and arthroscopic examination of the shoulder, and report it with review of literatures.