Exotropic-Duane Syndrome (XT-DRS) is a congenital cranial nerve dysinnervation disorder characterized by exotropia, anomalous head posture (contralateral face turn), limited adduction, globe retraction, palpebral fissure narrowing, upshoot and downshoot in adduction, and varying levels of limitation of abduction. Patients with XT-DRS often have poor convergence because attempted convergence induces co-contraction of the lateral rectus muscle (LR) due to anomalous LR innervation. We describe two patients with XT-DRS who underwent simultaneous lateral rectus muscle (LR) disinsertion and periosteal fixation (LRDAPF) and modified Nishida procedure (MNP) and discuss their outcome. In one patient, we combined this procedure with a contralateral LR recession. Anomalous head posture, exotropia, globe retraction, and palpebral fissure narrowing were reduced. Upshoot and downshoot were also reduced. Convergence improved in both patients.