INTRODUCTION: The surgical correction of anomalous movement such as upshoot in Duane syndrome is challenging. Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall is a new approach used to minimize or eliminate the effects of co-contraction including globe retraction, palpebral fissure narrowing and anomalous vertical movement. CASE PRESENTATION: We report a case of a 7-year-old boy who underwent this procedure for severe upshoot, globe retraction and exotropia in the left eye due to Duane syndrome. The patient achieved satisfactory ocular alignment following surgery. Upshoot and globe retraction were substantially improved. CONCLUSION: Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall is a safe and effective procedure for weakening of the anomalous lateral rectus muscle in Exotropic Duane Syndrome.