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Hemophagocytic lymphohistiocytosis (HLH) is a result of uncontrolled macrophage activation with prominent phagocytosis of platelets, erythrocytes, and lymphocytes and hematopoietic precursors. Attempt should be made to establish or rule out HLH in patients presenting with fever, multisystem inflammation, and varying degrees of unexplained pancytopenia. Owing to the guarded prognosis associated with HLH, differentiation from other causes of pediatric liver disease is critical and is a diagnostic challenge.

Liver biopsy to the aid in diagnosis of pediatric hemophagocytic lymphohistiocytosis: A case report