We report here a case of esthesioneuroblastoma an 11-year-old girl presenting as acute loss of vision with minimal evidence of orbital, nasal, or paranasal sinus disease, a rare presenting symptom for this tumor. The initial diagnosis was postviral optic neuritis, a pattern of presentation not previously reported. When vision failed to improve, magnetic resonance imaging revealed a lesion in the posterior ethmoid and sphenoid sinuses. After a biopsy, the tumor was excised through the cranium and paranasal sinuses. A mass completely surrounding the optic nerve without invasion was found. Histochemical staining suggested demyelination secondary to compression, confirming the clinical impression of optic neuritis. Anti-Leu 7 monoclonal antibody is useful in characterizing of this tumor, since other immunochemical stains can be misleading. Radiation and chemotherapy were given after the tumor was removed. Two years later, the patient has had neither recurrence nor complications.