EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) can have a high rate of morbidity and fatality, and earlier reports also proved that EBV infection could cause dilatation or aneurysm of the coronary arteries, but secondary ectasia accompanied with EBV-HLH had never been reported. This case warns us to pay more attention to the coronary artery changes in EBV-HLH in the future. © 2014 WIT Press.