Systemic lupus erythematosus is a chronic inflammatory autoimmune disease characterized by autoantibodies specific for highly conserved nuclear antigens, such as double-stranded DNA, histones and ribonuclear proteins. The course of the disease is unpredictable and nine times more common in females. Among the neurological manifestations of collagen vascular diseases is the most commonly recognized and better studied disease. Central nervous system lupus is a serious and potentially treatable illness presenting as difficult diagnostic challenge. This is a case report of a patient that suffered central nervous system manifestations of severe active lupus. Early recognition of this entity is essential to expedite appropriate to treatment and avoid future complications.