We present the case of a 14-year-old girl who was admitted to the hospital with the complaint of horizontal diplopia for 48 hours. Initially, she was diagnosed with idiopathic intracranial hypertension. During hospitalization she developed fever, macular facial rash, and chest pain, and because of abnormal laboratory findings the diagnosis of systemic lupus erythematosus was established. She received immunomodulatory therapy, a combination of corticosteroids, and intravenous infusions of the monoclonal antibody rituximab, which augmented her clinical improvement. Intracranial hypertension secondary to systemic lupus erythematosus is a rare manifestation, especially as a presenting symptom. In addition, the fact that the patient developed an aggressive form of systemic lupus erythematosus during the initial period of hospitalization for idiopathic intracranial hypertension is also uncommon. Moreover, to our knowledge, we are not aware of any published case reports of intracranial hypertension secondary to systemic lupus erythematosus that was treated with rituximab.