Duane's retraction syndrome (DRS) has been a recognized clinical entity for nearly a century. It is a clinically well described ocular disorder consisting of retraction of the globe with narrowing of the lid fissure in attempted adduction, frequent abduction deficiency with variable limitation to adduction, and upshoot and/or downshoot of the affected eye on adduction. Among strabismus patients the incidence of DRS is probably not more than 5%. Most cases are sporadic, but familial cases have been estimated at 10% by most authors. Numerous theories concerning the etiology and pathogenesis of DRS have been proposed, including agenesis of the abducens nucleus, but the majority of investigators concur that the characteristic findings are best explained by a paradoxical innervation of the lateral rectus muscle, which subsequently causes a cocontraction of the horizontal rectus muscles. The frequent association of DRS with other congenital anomalies suggests a teratogenic event occurring between the fourth to eighth week of gestation as an etiological factor. In this review historical aspects and theories of the syndrome are studied and statistical data are compiled and analyzed. Clinical features, differential diagnoses and variants of the syndrome are examined. Testing and treatment objectives are discussed.