Hepatic alveolar echinococcosis is an uncommon parasitic disease caused by Echinococcus multilocularis. It carries high fatality rates and poor prognosis if not diagnosed early and managed correctly. We report a 29-year-old female patient who admitted to our clinic with fatigue, weight loss and abdominal bloating in her right upper quadrant. Physical examination revealed the presence of a large palpable mass in her right upper quadrant, ascites and bilateral pedal edema. Computed tomography revealed a large non-homogeneous mass located in the right lobe of the liver and a dilated paraumbilical vein arising from the left branch of the portal vein. Echinococcus serology assessed by enzyme-linked immunosorbent assay was noted to be positive. Histopathologic examination of the hepatic mass was consistent with alveolar echinococcosis. The patient’s complaints were determined as being related to cirrhosis due to alveolar echinococcosis. Alveolar echinococcosis must be considered in patients with portal hypertension of uncertain etiology, especially in endemic regions.