Objective To summarize the clinical features of 201 cases with Duane's retraction syndrome (DRS) and discuss its differential diagnosis. Methods We retrospectively summarized the 201 cases from 1979 to 1996. The clinical features including chief complaints, sexual distribution, age at first visit, laterality, type of presentation, ocular deviation in the primary position, refractive errors, amblyopia, globe retraction, change of the palpebral fissure, upshoot and downshoot in adduction, binocular single vision, and its associated ocular and non-ocular anomalies were analysed. Results There were 99 males and 102 females with a female-to-male ratio 1:1. The 65.88% of DRS cases had left eye involvement with two-to-one predilection for the left eye. The most common form of the syndrome was type I (184 patients, 91.54%). Exotropia was the most common deviation in the primary gaze (72 patients, 35.8%). Among 118 patients, most had abduction deficits, globe retraction in adduction, and face-turn as to maintaining single binocular vision. Crocodial tears (26 patients, 11.93%) was the most frequently encounted ocular abnormalities. Conclusions Diagnosis of DRS in a typical case is not difficult, however, children with bilateral abduction deficits which may mimic DRS must be differentiated from the following four motility disorders, namely, abducens nerve palsy, Moebius syndrome, congenital oculomotor apraxia, and congenital or infantile esotropia.