Esthesioneuroblastoma (ENB) is a rare malignant tumor, which develops from the olfactory neuroepithelium and is one of the rarest tumors of the nasal cavity. Revelation by ocular signs is unusual. We report the original observation of a case of ENB of the olfactive placodes in a 28-year-old adult discovered during exophthalmia. The authors studied the clinical, radiological, pathological, and therapeutic aspects. ENB should not be forgotten in case of unilateral tumorous exophthalmia associating rhinological signs.