Hepatic alveolar echinococcosis is a rare but potentially life-threatening serious parasitic disease, developing as a result of intrahepatic growth of Echinococcus multilocularis larvae. It may present as an invasive mass causing biliary obstruction or portal hypertension due to invasion of the biliary and vascular structure of the liver. The only curative treatment of the disease is surgical resection; however, early diagnosis is the key point to achieve complete cure. In advanced disease, complications are common. We retrospectively analyzed 12 cases of hepatic alveolar echinococcosis and reviewed the relevant literature. A high index of suspicion and specific radiologic findings are very helpful in the diagnosis of the disease. While benzimidazole derivatives are used for the control of disease, endoscopic retrograde cholangiopancreatography is particularly effective in the biliary involvement, even in the late stage of disease.