Adult onset Still’s disease is a rare affection classified among non-hereditary autoinflammatory diseases. The clinical presentation goes from fever with arthralgia and maculopapular eruption to life-threatening manifestations such as secondary lymphohistiocytosis. The mechanisms of AOSD remains unclear, but seems implicate NK cells dysfunction with pro-inflammatory cytokines secretion including IL-1ß, IL-6 and IL-18 [1]. Several reports mentioned AOSD occurring during pregnancy, mostly in first and second trimester and there is still a debate on whether it could compromise or not the pregnancy outcome. We here report a case of AOSD revealed during pregnancy with a life-threatening presentation along with a review of 19 cases from literature.