Purpose: To report unilateral congenital cataract in a case of ipsilateral Duane retraction syndrome. Case Report: In this case, we present a six year old girl who was referred with ocular deviation. She had a history of congenital cataract surgery in the left eye at the age of two years. The subject had no associated systemic disease, developmental delay, or positive family history. She was finally diagnosed as having Duane retraction syndrome in the same eye. Conclusion: Duane retraction syndrome can be associated with congenital cataract due to the matching time of gestational development of the lens to that of ocular and nonocular anomalies associated with Duane syndrome. As both of these disorders are rare, coincidence of both in the same person and the same eye by chance is a very remote possibility.