Abstract
Systemic lupus erythematosus (SLE) is an autoimmune multisystem disease. Childhood-onset SLE is extremely rare and comprises only 10% to 20% of all cases. In this case report, we present a 9-year-old boy from northeastern India who presented with fever, cough, vague abdominal pain, lethargy and swelling of face and legs. Initial impression was one of sepsis with central nervous system (CNS) involvement and was treated accordingly. Detailed clinical examination with subsequent laboratory and imaging studies clinched the diagnosis of SLE. The patient showed rapid resolution of symptoms with immunoglobulins, cyclophosphamide and steroid therapy. A brief discussion on childhood neuropsychiatric lupus syndrome and SLE with CNS infections is included here.