ABSTRACT Background: Exotropia is uncommon in healthy children between 6 months and 1 year old. Methods: The charts of patients treated in our practice between January 1980 and August 1994 were reviewed for a diagnosis of infantile exotropia. All of them had an exodeviation during the first year of life. Children with neurologic defects, prematurity, trauma, craniofacial syndromes, or orbital abnormalities or ocular defects that would reduce vision were eliminated. Results: Sixty-six healthy patients with normal eyes had an exodeviation of 15 prism diopters (?) or more that persisted through the first year of life. Fifty-four were eliminated because the diagnosis had not been confirmed by a pediatric ophthalmologist before they were 1 year old or they had not been followed for more than 4 years. The 12 remaining patients were followed for at least 4 years (mean, 7.9 years). The mean age at the first examination was 7.8 months (range, 4 to 12 months). On early evaluation, exotropia was intermittent in four and constant in eight. Three had amblyopia. Ten required surgical correction. Exotropia was corrected with one operation in six patients; four required additional procedures, mostly to correct oblique muscle overaction and dissociated vertical deviation (DVD). At the most recent visit, all 12 patients had equal visual acuity in both eyes and satisfactory ocular alignment. Five had fusion at distance and near, but only two had stereo acuity of 100 seconds of arc or better. Conclusion: Infantile exotropia is rare. But, like patients with infantile esotropia, those with exotropia can be expected to have good visual acuity but unstable ocular alignment. Also, as with early-onset esotropia, although surgical intervention is usually required, high levels of binocular function develop in some patients with exotropia. Oblique muscle overaction and DVD are common, often becoming manifest by the first year of age. Unlike infantile esotropia, nystagmus is rare.