Esthesioneuroblastoma (ENB) is a rare malignant tumor accounting for 3% of all sinonasal cancers. It arises from the olfactory epithelium and usually affects subjects aged 30-50 years. It is uncommon in children. It is often diagnosed late because tumor remains confined to its original site for long and prognosis depends on locoregional extension (in particular to the brain and the orbital regions). We report the case of a 3-year old child with sphenoidal esthesioneuroblastoma discovered after early onset blindness. This study aims to highlight the clinical, radiological, anatomopathological, therapeutic and prognostic peculiarities of this disease while insisting on the importance of early diagnosis affecting prognosis. Unfortunately, diagnosis is still pejorative due to high recidivism rates as well as to the occurrence of distant metastases (in particular lung and bone metastases).