Alveolar echinococcosis (AE) is a zoonotic disease that is caused by Echinococcus multilocularis that affects liver and a variety of organs and tissues. It differs from other echinococcal disease because it shows tumor like behavior in the affected organ and tissues. The treatment of choice is concomitant medical therapy and resection with negative margins. Nevertheless, resection with the intent of negative margins (R0) may lead to serious complications such as liver failure. In the present case report, we used Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) procedure, which was defined in 2012 by Schnitzbauer et al, in a 28-year-old male patient to avoid complications of major liver resection in order to treat alveolar echinococcosis. Until now, we have not encountered any study using ALPPS procedure for the treatment of alveolar echinococcosis. In the present case report we aimed to show that ALPPS procedure can be safely performed for margin-negative resection of primary or recurrent AE that shows a tumor like behavior. It is our opinion that this procedure should be performed in centers that have expertise and sufficient technical capacity to perform liver transplantation and advanced liver surgery.