Olfactory neuroblastoma (ONB) is an uncommon malignant tumor of neural crest origin, which commonly arises in the superior nasal cavity. Ectopic origin of an ONB is exceedingly rare. We describe a rare case of an ectopic sphenoclival ONB with extensive involvement of the central skull base and with development of systemic metastases. To our knowledge, ours is the first case that describes the imaging features of this rare entity on computed tomography (CT), magnetic resonance imaging, 18 F-fluorodeoxyglucose-positron-emission tomography/CT, and digital subtraction angiography. We also describe the histological features, imaging differentials, and treatment options for this tumor along with a brief literature review.