The authors present a case of an optic nerve hemangioblastoma in a young woman with von Hippel-Lindau disease. The initial diagnosis was made by incisional biopsy. Tumor growth led to progressive proptosis and loss of light perception. Excision was carried out by lateral orbitotomy. Clinically and radiographically, the tumor resembled an optic nerve meningioma or glioma. Review of the other known cases offers no information as to the potential spread of this benign tumor from the intraorbital optic nerve to the optic canal. Optic nerve hemangioblastoma must be considered in the differential diagnosis of optic nerve tumors in patients with or without von Hippel-Lindau disease.