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Hemophagocytic syndrome remains a rare but life-threatening complication and is associated with intensive care unit (ICU) admission. The pathophysiology is based on a defect of cytotoxicity in T cells that results in a state of hyperinflammation in the presence of a trigger. As a consequence, patients may develop multiorgan failure. The diagnosis of hemophagocytic syndrome (HS) remains difficult and relies on persistant high-grade fevers in the absence of infection and on constellation of laboratory parameters. However, prompt diagnosis and treatment (supportive care and specific treatment) are associated with improved outcome. Interaction with other specialists (hematologist, internist) may improve the diagnosis and treatment strategy.This article describes diagnostic tools, organ failures associated with HS, main etiologies, and management.