Systemic juvenile idiopathic arthritis (sJIA) is the most frequent rheumatic disease complicated by macrophage activation syndrome (MAS), which can be life-threatening in children. Current animal models show only some of the characteristics of sJIA and MAS, and there is no perfect animal model that can replicate the whole process of sJIA and MAS. In this paper, the clinical features, advantages and shortcomings of the currently proposed animal models of sJIA and MAS are reviewed, aiming to provide ideas for exploring better-represented animal models of sJIA and MAS.