Mediterranean spotted fever (MSF) is a tick-borne acute febrile disease caused by Rickettsia conorii and characterized by fever, a maculo-papular rash and a black eschar at the site of the tick bite. We describe the case of a 3-year-old boy with MSF who developed a transient right coronary artery ectasia. The patient was brought to the hospital after four days of fever and mild myalgia of the legs. The suspicion of MSF arose due to the presence of a maculo-papular skin rash and treatment with oral clarithromycin was started. After four days fever persisted and the differential diagnosis of Kawasaki syndrome was considered. Echocardiography showed a dilated right coronary artery with hyperreflective walls. Treatment with intravenous immunoglobulin was initiated while clarithromycin was continued. After one day the fever disappeared. An immunofluorescent antibody test performed after four weeks confirmed a R. conorii infection. A follow-up echocardiography was normal six weeks and six months later. We suggest that ectasia of the coronary arteries may be a manifestation of rickettsial vasculitis. Prospective studies are needed to understand the frequency and the possible consequences of this phenomenon in the course of MSF.