OBJECTIVE Monocular blepharospasm (MBLS) is precipitated by ipsilateral ocular pathology and becomes bilateral over time. Two patients are reported with monocular visual disturbance exhibiting monocular involuntary eyelid closure (MIEC), resembling MBLS. METHODS A 31-year-old female with hyperthyroidism developed monocular polyopia with left eye vision on binocular fixation followed by ipsilateral visual extinction. Monocular vision was otherwise normal. A 57-year-old male with renal failure developed monocular blindness secondary to retinal hemorrhage. RESULTS The first patient had anisocoria and proptosis. Brain magnetic resonance (MR) was normal. The second patient had involuntary movements of the left eye. Head computerized tomography (CT) was normal. Both patients exhibited MIEC of the eye with visual impairment that could be overcome voluntarily, but reappeared upon distraction. Neither of the two developed BLS in long-term follow-up. CONCLUSIONS MIEC represents a complication of ipsilateral ocular pathology in the absence of other involuntary movements, apraxia of eyelid opening, or blepharospasm (BLS). MIEC needs to be distinguished from MBLS, since not all MBLS becomes bilateral.