Abstract:The incidence of hydatid disease of the liver caused by Echinococcus granulosus in Austria, a nonendemic region in central Europe, is documented over a 10-year period. Since 1984, 28 patients (24 women, 4 men) with hydatid disease of the liver have undergone surgery in our department. A definitive diagnosis was established by combining serological tests (an enzyme-linked immunosorbent assay with Echinococcus multilocularis antigen and an indirect hemagglutination assay with Echinococcus granulosus antigen) and abdominal imaging (computed tomography scan and ultrasonography). Twenty-four patients (85.7%) had uncomplicated echinococcosis; in most of them (16) total closed pericystectomy was done. Four patients (14.3%) had complicated echinococcosis, suffering from intrabiliary rupture and spontaneous perforation into the peritoneal cavity, and in two of these patients (7.1%) pyogenic transformation, one with abscess perforation, Occurred. Postoperative complications were significantly more common in patients with complicated echinococcosis and included two bile leaks (necessitating prolonged tube drainage), pleural effusion, atelectasis, and wound infection. One recurrence of hydatid infection due to previous spontaneous perforation required reoperation 4 years after the primary event. The low recurrence rate of uncomplicated echinococcosis (0%) over a mean period of 5.6 years and of 3.6 % when peritoneal echinococcosis was included may be the result of the radical surgical technique employed.