Nasal signs and symptoms commonly accompany cephalgia in some headache syndromes. Head pain associated with sinusitis is also fairly well-recognized. However, referred cephalgia of rhinogenic origin, in the absence of sinonasal symptoms or disease, is poorly understood. We report a case of a patient with chronic and severe headache for whom neurological and neurosurgical evaluation failed to reveal an etiology, but who possessed an intranasal anatomical variant (without sinusitis). Her headache resolved after endoscopic nasal surgery. We discuss the mechanisms of such referred pain, and the recent technological advances that made the diagnosis and treatment of this disorder possible.