Congenital absence of long head of biceps is an uncommon condition. We report the first case of bilateral congenital absence of long head of biceps tendon in a young male presenting with recurrent anterior right shoulder dislocation associated with an ipsilateral congenital radial head dislocation. This association has not hitherto been described. We present the clinical, imaging and arthroscopic findings, as well as a review of literature to promote awareness of this rare pathology and discuss its role in joint instability and associated congenital abnormalities.