The management of children with oculomotor nerve palsy is complicated by their variable presentation, amblyopia, potential loss of binocularity, and associated neurological disease. Our purpose was to evaluate the causes, neurological associations, treatment, and sensorimotor outcomes of a group of children who developed oculomotor nerve palsy.We identified 18 children aged 14 years or younger, of whom 13 (72%) were less than 8 years old, through a retrospective record review of all children with oculomotor nerve palsy seen between January 1995 and January 2001 by one of the authors (C.J.L.).Congenital-onset oculomotor nerve palsy was the most frequent presentation, followed by traumatic, neoplastic, vascular, and migrainous or para-infectious etiologies. Pupil sparing was seen in 1 patient with neoplastic etiology. Primary aberrant regeneration was the presenting sign in a child with neurofibromatosis type 2. Amblyopia developed in 7 (39%) children and was successfully treated in 5 of the 7 (71%). Stereopsis was maintained in 6 (33%) children of whom 2 were within the amblyogenic age group. Six (33%) patients underwent strabismus surgery, and 3 of them (50%) achieved orthotropia and maintained stereopsis.Children with oculomotor nerve palsy require neuroimaging. Their pupillary signs, unlike those of adults, are not helpful in differentiating compressive etiologies from other causes. Good visual acuity was obtained in children within the amblyogenic age group with appropriate occlusion therapy. Stereopsis was uncommon in children who developed third nerve palsy during the amblyogenic period; preservation of stereopsis was dependent either on rapid and complete recovery, or on the child's adoption of a compensatory head position.