Aim: Esthesionevroblastoma is a rare malignant neuroectodermal tumor that begins development of neuroepithelial cells of the olfactory membrane. Therefore the biology of the tumor, surgery is a method of choice. Endoscopic transnasal method with craniobasal resection is favorable alternative to more invasive surgical methods in a rigorous assessment of patients. Our aim is to describe the first case in the country operated endonasal with typical clinical and histopathological findings of olfactory neuroblastoma, and to discuss opportunities for radical surgery by endoscopic endonasal sinus surgery. Material and methods: We present a patient with nasal obstruction, epistaxis, smell disturbance, headache and eye symptoms. Results: Single step transnasal endoscopic resection with craniektomia, then undergoes postoperative radiotherapy is made on the patient. One year after surgery, no clinical, endoscopic and radiologic evidence of disease recurrence. Conclusion: Esthesionevroblastoma is a rare malignant tumor that untypical because its initial symptoms are often diagnosed at later stages. Early detection and timely performed treatment is important moment for the prognosis.