Systemic lupus erythematosus (SLE) involves dysregulation of the immune system, consequently affecting multiple organ systems, including the cardiovascular, neuropsychiatric, renal, and musculoskeletal systems. Optic neuritis and intracranial hypertension are conditions that rarely occur in SLE, and their coexistence has not been reported to date. Herein, we report the first case of a patient who was diagnosed with SLE complicated by concurrent intracranial hypertension and bilateral optic neuritis. An 11-year-old Thai girl had a low-grade fever, discoid rash, oral ulcer, chronic headache, and fluctuating diplopia. She experienced bilateral vision loss just before presentation. She was diagnosed with juvenile SLE. We believe that her headache, which was probably a symptom of optic disc edema, was due to intracranial hypertension. Furthermore, she exhibited vision loss and color vision deficit and was diagnosed with bilateral optic neuritis. Her condition improved on treatment with corticosteroids (intravenous pulse methylprednisolone for 3 days, followed by 1 mg/kg/day oral prednisolone tapered over 3 months). The occurrence of optic neuritis and intracranial hypertension during an active SLE inflammation and a rapid response to high-dose corticosteroids support the fact that SLE was the etiology of these neuropsychiatric conditions. Early diagnosis and prompt treatment in such cases can lead to favorable outcomes.