Hemophagocytic syndrome (HPS), whether familial or acquired, is a clinico-pathological entity, characterized by uncontrolled hyper-inflammation, leading sometimes to a lifethreatening condition. Liver dysfunction is central in HPS: hepatomegaly is discovered in half cases, liver enzyme elevation and/or cholestasis are very frequent, cholestasis is a prognostic factor, and liver biopsy is often necessary to confirm HPS and to diagnose the underlying disorders in secondary HPS. The spectrum is large from acquired SAM than can be cured with the treatment of his trigger, to genetic cases only cured by stem cell transplantation.