Congenital esotropia represents the most common type of strabismus. Its pathogenesis, however, remains uncertain. It is typically characterized as a large angle, constant esotropia with onset during the first six months of life. Associated clinical findings include normal refractive errors for age, amblyopia, dissociated vertical deviation, inferior oblique muscle overaction and nystagmus. It must be distinguished from Duane's retraction syndrome, Moebius syndrome, nystagmus blockage syndrome, and early onset accommodative esotropia, as well as other causes of esotropia in infancy. The surgical management may involve recession of both medial recti muscles, unilateral recession of a medial rectus muscle and a resection of a lateral rectus muscle or three or four muscle surgery.