Epstein-Barr virus (EBV) infection is a common viral illness typically presenting with symptoms such as fever, sore throat, and lymphadenopathy. Hepatic involvement in EBV infection is usually mild and transient. However, severe cholestatic liver disease due to acute EBV hepatitis is rare, especially in young adults. Secondly, hemophagocytic lymphohistiocytosis (HLH), the abnormal activity of lymphocyte function leading to hemophagocytosis and multi-organ failure, is a rare complication of EBV. In the context of Los Angeles County, the locale of this study, the incidence of secondary nonfamilial HLH among patients over the age of 15 is reported at 0.9 cases per million annually, with epidemiological data specific to EBV-associated HLH even more notably limited. We report a case of a 20-year-old female patient presenting with fever and chills, ultimately diagnosed with acute EBV hepatitis causing severe cholestatic liver injury, with concurrent positive antimitochondrial antibody and HLH, without multi-organ failure, who showed significant improvement with the administration of N-acetylcysteine (NAC), highlighting its potential therapeutic role in EBV-associated liver diseases.