BACKGROUND: Cavernous malformations consist of dilated vascular spaces separated by fibrous tissues that create a well-defined mass. They are increasingly recognized in the brainstem with the advent of magnetic resonance imaging (MRI). This study was aimed to understand neuro-ophthalmologic findings in the brainstem cavernous malformations. METHODS: Fourteen patients with brainstem cavernous malformations, who presented mainly with neuro-ophthalmologic findings, were asked to participate in this study. The diagnosis was based on the characteristic MRI findings of well-circumscribed lesions with a mottled core of mixed-signal intensity. All patients received full neuro-ophthalmological evaluation, including oculography in some of them. RESULTS: Seven patients mainly presented with ophthalmoplegia, 3 with abducens palsy, 1 with oculomotor palsy, 1 with gaze-evoked nystagmus, and another 2 with internuclear ophthalmoplegia. In the other 7 patients, the main neuro-ophthalmologic findings were ocular dyskinesia with variable degree of ophthalmoplegia. Six of them had oculopalatal tremor and the other patient showed macrosaccadic oscillation. Six of the 14 patients had a history of recurrent ophthalmoplegia from repeated bleedings of the cavernous malformations. CONCLUSIONS: Cavernous malformations in the brainstem can manifest with various ocular motor abnormalities. In patients with recurrent ophthalmoplegia or oculopalatal tremor, cavernous malformation in the brain stem should be considered in the differential diagnosis.