Background: Significant progress in managing alveolar echinococcosis (AE), Echinococcus multilocularis infection, has been reported in recent years.Case Report: We present a fatal case of AE to reaffirm that E. multilocularis infection remains a life threatening disease that requires optimal medical and surgical therapy.A 33 year old male was admitted to hospital service complaining of pruritus. On physical examination, icterus and hepatomegaly were noted. Biochemical tests revealed hyperbilirubinemia - 37.6 µmol/L, elevated alkaline phosphatase - 1330 U/L and gammaglutamylotransferase -126 U/L. Abdominal ultrasonography revealed a 126 x 88mm mass in the right liver lobe with mixed echogenicity. Abdominal CT scan confirmed the mass that contained areas of liquid andcalcifications. Fine needle biopsy failed to reveal neoplastic cells. Indirect haemagglutination for E. granulosus/multiloculariswas positive (titer 1:200) and albendazole, 800mg daily was initiated. Subsequent endoscopic retrograde cholangiopancreatogram (ERCP) revealed common duct narrowing and enlarged bile ducts in the left lobe. The patient received a total of two months of albendazole. Subsequently patient was admitted to Surgery where a partial resection of liver lession was performed. The postoperative course was complicated by bile leakage and a prosthesis was inserted. During the following ten months the patient was hospitalized for ERCP procedures to change the prosthesis. Twenty two months after the initial diagnosis, he was admitted for liver failure. Radiological immaging techiques confirmed progression of thedisease. The patient died 30 months following the diagnosis.Conclusions: We would like to emphasize that physicians and surgeons must work closely together in the diagnosis and therapy in alveolar echinococcosis patients.

Keywords: Echinococcus multilocularias, alveolar echinococcosis, Infection