We report the case of a 60-year-old woman in whom migraine with typical aura heralded the presence of an occipital lobe tumor. Her headache was characterized by recurrent episodes of visual aura confined to the left visual field followed by right hemicranial throbbing headaches accompanied by nausea, photophobia, and phonophobia. Interictal neurologic and ophthalmologic examinations were negative, as was an unenhanced brain CT scan. The headaches increased in frequency over 4 months despite a number of medications known to prevent attacks of migraine. A low-grade right occipital lobe tumor was eventually discovered on MR scan. This case illustrates that headache fulfilling the International Headache Society (IHS) criteria for migraine with typical aura can occur in association with an occipital lobe tumor.