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A 56-year-old male was admitted with an acute headache and sudden ptosis on the right side. No ophthalmological or neurological etiologies were apparent. A mucocele of the right posterior ethmoid sinus was observed with radiology. After the marsupialization of the mucocele via a transnasal endoscopic approach, the patient’s symptoms (oculomotor nerve paralysis and headache) resolved in 4 weeks. Oculomotor paralysis is a rare symptom of an ethmoidal mucocele. In this article, we report this rare case along with a literature review.