Systemic lupus erythematosus (SLE) is a diagnostically challenging autoimmune multisystem disease with intracranial hypertension as a rare initial clinical manifestation. This is a case report of a 27-year-old woman with a prior history of psychogenic non-epileptic attacks, intracranial hypertension, headache, visual impairment, papilloedema, and a BMI of 24 kg/m2. Upon acetazolamide treatment for intracranial hypertension and before the diagnosis of SLE was reached, the patient developed respiratory distress and metabolic acidosis, due to underlying SLE glomerulonephritis.