Esthesioneuroblastoma is a rare malignant neoplasm of oflactory neuroepithelium and usually located at the olfactory cleft at superior nasal cavity. Ectopic localization of esthesioneuroblastoma is even rarer and usually posed with a diagnostic dilemma and delay in the diagnosis and management, We report a rare case of ectopic esthesioneuroblastoma of the sphenoclivus with the presentation of intermittent unilateral epistaxis, intermittent intractable headache without anosmia. Nasal endoscopy findings showed a pulsatile mass at the anterior face of the sphenoid sinus with extension posteriorly towards the clivus region and occupying the floor of the sphenoid sinus. Endonasal transclival endoscopic excision of tumour was performed which involved otorhinolaryngology surgeon and neurosurgeon with intraoperative navigation imaging and frozen section. The histopathological findings was esthesioneuroblastoma. Due to its rarity and unusual presentation, the diagnosis of ectopic esthesioneuroblastoma is difficult and can be misdiagnosed with the other type of malignancy. Therefore, the histopathological result is important in confirming the type of tumour and can lead to the next step of management.