PURPOSE: To report four patients with isolated comitant esotropia and Chiari I malformation and discuss the most appropriate management.

METHODS: Case reports and literature review.

RESULTS: All four patients (5, 14, 16, and 37 years of age) presented with an isolated comitant esotropia that led to the diagnosis of Chiari I malformation. The first two patients underwent uncomplicated neurosurgical decompression of their malformation, followed by complete resolution of their esotropia. The third patient underwent strabismus surgery and experienced initial resolution of the esotropia, but eventual recurrence resulted in the strabismus surgery being repeated 5 years later. The fourth patient had strabismus surgery with resolution of the esotropia but only 2 months of follow-up.

CONCLUSION: Although management of patients with Chiari I malformation and severe neurologic findings typically includes surgical decompression, management is less straightforward in cases with subtle findings or in which ocular findings are isolated. The decision to perform neurosurgical decompression or strabismus surgery should still be made on a case-by-case basis, with the understanding that strabismus surgery may provide only temporary ocular alignment.