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Macrophage activation syndrome is a potentially fatal complication of systemic jeuvenile idiopathic arthritis, our novel understanding of the unique pathogenesis of both diseases has enabled the physicians to approach the condition with different aims, we now not only want to relief symptoms but we do aim to cure our patients. Recent drugs are now available with much improvement in the prognosis. Here we discuss systemic jeuvenile idiopathic arthritis and its complication macrophage activation syndrome with reference to our case report.