Introduction: Systemic juvenile idiopathic arthritis (SJIA) is characterized by fever, rash, arthritis, lymphadenopathy, hepatosplenomegaly, and serositis. It is frequently considered an autoinflammatory syndrome. There is interplay between different pro-inflammatory cytokines in SJIA pathogenesis. Sufficient control of inflammation is crucial to prevent complications. Treatment strategies in SJIA have changed dramatically with the introduction of increasing number of biologic agents. These modern drugs have improved the inflammation control and reduced side effects of traditional long-term treatments.