Alveolar echinococcosis (AE) is a rare but potentially serious zoonosis for which an early diagnosis is of primary importance. We report the first observation of AE in a cardiac transplant patient infected by hepatitis C virus. He first presented with a single asymptomatic hepatic nodule. The liver biopsy showed an epithelioid granuloma with necrosis. We review the clinical features, diagnosis and outcome of this disease in immunocompromised hosts. In immunocompromised patients living in areas endemic for Echinococcus multilocularis, AE should be included in the differential diagnosis of tumor like lesions of the liver.