Esthesioneuroblastoma is a rare malignant neoplasm arising in the nasal cavity. Huge esthesioneuroblastomas, extending into the ethmoid roof and the cribriform plate, or invading the anterior cranial fossa, have traditionally been treated by craniofacial resection. Because of the invasiveness and potentially disfiguring results of a transfacial approach, a new technique has been proposed, combining endoscopic nasal and anterior craniotomy resection. We describe the case of a young male presenting with a large esthesioneuroblastoma involving the nasal cavity and the anterior cranial fossa, causing a huge skull base destruction. A combined transcranial and endoscopic nasal resection resulted in a macroscopically total removal of the tumor. The operative technique is reviewed in detail, along with the method used for the reconstruction of the anterior skull base defect.