Abstract: Esthesioneuroblastoma(ENB)is an uncommon malignant tumor of the nasal vault.With only about 1000 cases reported in the world.These tumors have distinctive clinical, radiographic, histologic, immunohistochemical and molecular features.The best treatment has yet to be defined.Bicranial-facial surgical approach remains the primary treatment for ENB.But for its technical complexity, the treatment effect is still not satisfied.This manuscript will review the clinical and pathologic feature, current treatment of ENB focusing on the recent advances in these tumors.