Abstract
Hemophagocytic lymphohistiocytosis (HLH) is characterized by an uncontrolled activation of immune cells. HLH can be primary, due to a genetic predisposition, or secondary, due to an underlying disease such as systemic juvenile idiopathic arthritis (sJIA). Primary or secondary HLH and sJIA present similarly. Clinical and biochemical parameters can be used to differentiate between them. In patients with a persistent fever, it is important to consider an underlying immune and/or rheumatologic problem. We present the case of a seven-month-old girl who presented with symptoms of HLH and was later diagnosed with underlying sJIA.