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 experimental 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. This article reviews the clinical features, advantages and shortcomings of these models, with the aim of providing ideas for exploring more representative animal models of sJIA and MAS.