Hemophagocytic lymphohistiocytosis is a rare and often fatal disease that may occur in solid organ transplant recipients. Here, we describe 2 patients who developed hemophagocytic lymphohistiocytosis after having a lung transplant and present a review of all cases of hemophagocytic lymphohistiocytosis occurring in solid organ transplant recipients. Diagnosis of hemophagocytic lymphohistiocytosis relies on the association of clinical findings and the presence of hemophagocytosis. Clinical presentation is nonspecific and patients may present with unexplained sepsis or multiple organ failure. Management consists of treating the underlying process; but unfortunately, the prognosis is poor. © Başkent University 2011 Printed in Turkey. All Rights Reserved.