The development of an esthesioneuroblastoma outside to the region in which olfactory epithelium exists is extremely rare. Only nine cases were reported in the previous literatures. The author presents a 40 years-old man with ectopic esthesioneuroblastoma in sella turcica. In contrast to the previous nine cases, our case presented unusual presentations-CSF rhinorrhea and meningitis. Endoscopic transphenoid approach with removal of tumor and repair of dura defect followed by radiotherapy offered a good result in this case. Moreover, differential diagnosis and the origin of the ectopic esthsioneuroblastoma would be discussed.