Purpose: We report a case of cavernous sinus meningioma presenting with unilateral pseudo-internuclear ophthalmoplegia.Case summary: A 50-year-old female visited our clinic with a history of insidious onset of horizontal diplopia at primary and left gaze over 3 weeks. The symptom had diurnal variation and was aggravated with fatigue. There was intermittent upper eyelid drooping of the right eye. She had a history of migraines, which had recently worsened. The pupils showed a normal response to light and near stimulation in both eyes. The extraocular examination showed 14 prism diopter (PD) exotropia at primary, and 25 PD exotropia at left gaze, with -1 adduction limitation of the right eye. There was abducting nystagmus of the left eye. Ocular myasthenia gravis was suspected based on the clinical findings. Magnetic resonance imaging of the brain revealed a right cavernous sinus meningioma. The ocular symptoms improved after radiotherapy. At 3 months of follow-up, the ocular motility findings had improved to 4 PD exotropia, without adduction limitation of the right eye. The upper eyelid drooping had improved.Conclusions: A cavernous sinus meningioma can be manifested by various types of ocular motility disorder, including pseudo-internuclear ophthalmoplegia.