PURPOSE To describe the clinical features and surgical outcomes of patients with unilateral exotropic Duane retraction syndrome (DRS). METHODS The medical records of patients with unilateral exotropic DRS who underwent surgery between March 2015 and February 2018 were reviewed retrospectively. RESULTS A total of 40 patients (mean age, 18.75 +/- 12.54 years; 21 males [53%]) were included. In 28 patients (70%) the left eye was involved. All patients had globe retraction and head turn toward the opposite side of the affected eye. Remarkable upshoot/downshoot movement was detectable in 11 patients (28%). The mean deviation for near and distance improved from 24.37(Delta) +/- 12.34(Delta) (range, 6(Delta)-77(Delta)) and 19.67(Delta) +/- 10.76(Delta) (range, 4(Delta)-60(Delta)) to 4.25(Delta) +/- 8.61(Delta) (range, 0(Delta)-50(Delta)) and 2.62(Delta) +/- 6.15(Delta) (range, 0(Delta)-35(Delta)) after the first surgery (P <0.001 for near and far deviation). Mean postoperative follow-up was 7.82 +/- 9.45 months. Two patients required reoperation. Different types of surgeries, including lateral rectus recession (with or without Y-splitting), lateral and medial rectus recession (with or without Y-splitting of the lateral rectus muscle), bilateral lateral rectus recession, and lateral rectus recession with vertical rectus nasal transposition were performed. Twenty-four patients (60%) were successfully treated with only a single recession of the ipsilateral lateral rectus muscle. The mean lateral rectus recession was 7.45 +/- 0.73 mm (range, 6-8.5 mm), and the mean dose-response for lateral rectus recession was 2.79(Delta) +/- 0.64(Delta)/mm for near and 2.45(Delta) +/- 0.67(Delta)/mm for distance. CONCLUSIONS Simple lateral rectus recession (with or without Y-splitting), even in the presence of significant deviation (through 35(Delta)), seems to be a successful procedure for management of patients with unilateral exotropic DRS.