We present a case of Ehrlichia-induced hemophagocytic lymphohistiocytosis (HLH), which resulted in a fatal outcome. Hemophagocytic lymphohistiocytosis is a rare syndrome of abnormal activation of the cellular immune system, which can be potentially fatal. Infections are a common trigger of the secondary form of the syndrome. We review the literature for published reports of Ehrlichia-induced HLH, all of which have resulted in a favorable outcome. We try to analyze the reasons for the poor outcome in our case including the possibility of central nervous system involvement with HLH. Early recognition and treatment of HLH and its associated central nervous system involvement may improve outcome.