A 16-year-old female with fever was admitted to our hospital. On admission, her serum IgM antibody against Epstein-Barr virus (EBV) was positive. Then, the disease aggravated and acute kidney injury occurred gradually. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) was confirmed by serum test and kidney biopsy. The patient was treated with oral methylprednisolone. Along with the disappearance of the IgM anti-EBV antibody, the AAV also relieved without relapse during follow-up for half a year. Although a previous study indicated that there was a high positive rate of ANCA in the sera positive for IgM antibodies to EBV and EBV infection might trigger the relapse of AAV, this is the first case of incipient AAV associated with acute EBV infection. One possible explanation might be that EBV infection stimulated the production of ANCA.