Nasopharyngeal carcinoma (NPC) is a rare type of cancer that is one of the most challenging cancers to diagnose correctly at the initial phase because of the different irrelative symptoms. Headache per se is rare and maybe a misleading symptom for diagnosing NPC. We report a case of a 37-year-old Saudi civil servant male with NPC who presented to the clinic complaining of a continuous, dull occipital headache that is progressively worsened over the last three months, not responding to over-the-counter analgesics. Computed tomography revealed a large ill-defined infiltrative heterogeneously enhancing soft tissue mass that obliterated the fossae of Rosenmuller and pharyngeal openings of both Eustachian tubes. The histopathological diagnosis was undifferentiated non-keratinizing NPC that is positive for Epstein-Barr virus. As in this case, headache alone can be the sole presenting symptom for NPC. Therefore, physicians should think broader in such a presentation to appropriately diagnose and treat NPC.