Abstract We report the case of a 14-year-old girl who suffered from headache, fatigue, caught and a fever with body temperature up to 40 °C for a week. The patient had abdominal enlargement and clinical examination showed the hepatomegaly +5 cm. In ELISA assays employing Em2+ antigen, high titres of specific IgG antibodies to Echinococcus multilocularis somatic antigens were found in the serum. Western blot analysis also indicated on presence of alveolar echinococcosis (AE). CT examination showed expansive foci in the right liver lobe and histological examinations of the biopsy after PAS staining confirmed the presence of alveolar echinococcosis. After 320 days of chemotherapy with albendazole, CT revealed progression of parasitic foci and new abscess lesions inside parasitic cysts were detected, with some parts being necrotic. The surgical removing of foci was performed as following — the laparotomic revision, the removal of the right liver lobe with dilatation, reconstruction of v. cavae cum prothesis PTFE in length 12 cm. Three months after the operation, the rest of the patient’s left liver lobe had grownup and compensated at least a part of the removed liver.