Pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying Iesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. Pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in Korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. Headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache.