Hemophagocytic lymphohistiocytosis (HLH) is one of the complications of Epstein-Barr virus (EBV) infection. Although the patients who have developed HLH following EBV have normal immune system, there are a few patients with EBV-induced immune deficiency who develop HLH as well. Here, we describe the case of a 10-year-old girl with neurological complications caused by EBV-induced HLH. The patient received rituximab, leading to weakening inflammation associated with EBV infection and suppression of disease through quick treatment.