Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory neuroepithelium. ENB constitutes only 3% of all malignant intranasal neoplasm. Because of the rarity, the number of patients of ENB treated in individual departments is small. Most of these patients presents in locally advanced stages and require multimodality treatment in form of surgery, chemotherapy and radiotherapy. Multimodality approach with a risk-adapted strategy is required to achieve good control rates while minimizing treatment related toxicity.