We describe a 59-year-old female with severe anticonvulsant hypersensitivity syndrome (AHS) associated with Epstein-Barr virus (EBV) infection. The causative drug was speculated to be carbamazepine. Recurrent EBV infection was demonstrated by the presence of anti-EBV early antigen IgM antibodies and anti-EBV nuclear antigen IgG antibodies. To our knowledge, only one case of drug hypersensitivity syndrome (DHS) associated with EBV has been reported in the English-language literature. Our case is the second report of EBV-associated DHS, which suggests that EBV infection may contribute to the pathogenesis of AHS in a few patients.