BACKGROUND: Pneumatized anterior clinoid process is a common anatomic variant. Mucocele formation is a known complication of clinoid drilling during certain intracranial operations; however, mucoceles of pneumatized anterior clinoid processes have been found to spontaneously occur. CASE DESCRIPTION: A 44-year-old male presented with complaints of left-sided retro-orbital pain, double vision, and numbness over the upper face and scalp on the left side of 1-week duration. On examination, he was found to develop cranial nerve III, IV, and VI palsies with pupillary sparing, ophthalmic division cranial nerve V dysfunction, and eventually, the onset of vision loss. CONCLUSIONS: We report a case of spontaneous anterior clinoid process mucocele presenting with orbital apex syndrome. This was treated successfully with anterior clinoidectomy for decompression.