Mucoceles arising from the anterior clinoid is rare. Although intrinsically benign, mucoceles in the sphenoid sinus (anterior clinoid variant) can lead to complications due to their proximity to other structures. We present a case of a young man whose visual complaints were typical for retrobulbar optic neuritis. Results of radiologic tests for diagnosis led us to the early detection of a surgically treatable condition, a sphenoid sinus mucocele (anterior clinoid variant). Early surgery was helpful in ameliorating the symptoms, leading to full recovery of visual function. The clinician should be aware of this rare entity with its presentation in a wide variety of signs and symptoms.