A 32-year-old male presented with acute left vision loss during a second recurrence of optic neuropathy. Steroid pulse therapy had been effective in both the first episode 9 years previously and the first recurrence 5 years previously. Magnetic resonance imaging demonstrated an anterior clinoid process mucocele compressing the optic nerve. Although surgical treatment was performed, improvement was limited. This report indicates that steroid pulse therapy could be an alternative treatment to obtain temporary remission, but surgical treatment should be considered to prevent irreversible neurological deficits. This paper also presents a review of the literature on anterior clinoid process mucoceles.