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Headache is a frequent occurrence in patients with systemic lupus erythematosus (SLE). We present two cases of young women with headache and papilledema. One patient had this as a presenting manifestation of SLE, and the other had known active SLE. The first patient had a diagnosis of cerebral venous thrombosis, and the other pseudotumor cerebri. Both of these conditions are rare but have known associations with lupus. In the lupus patient with headache, it is essential that the clinician rule out these potentially treatable conditions by carefully taking a history and performing a physical examination as well as using ancillary investigations, including lumbar puncture, computed tomograph and magnetic resonance imaging as required. These uncommon causes of headache in the lupus patient must not be missed, because there is significant potential for morbidity, such as visual loss, or even mortality.