Radical resection is the first choice for hepatic alveolar echinococcosis (HAE). However, many patients with advanced HAE have no chance to be treated with curative resection owing to the long clinical latency. This study aimed to evaluate the necessity of aggressive operations, like palliative resection and orthotopic liver transplantation (OLT), in the management of advanced HAE. A retrospective study analyzed 42 patients with advanced HAE treated with palliative resection (N = 15), palliative nonresective procedures (N = 13), OLT (N = 3), or albendazole therapy alone (N = 11). The patients' condition before treatments was comparable among all the four groups. The overall 1-year, 3-year, and 5-year survival rates of the 42 cases were 81.0%, 45.2%, and 23.8%, respectively. No event occurred to end the follow-up during the 5-year observation period except death. The survival time (median ± standard error) of the palliative resection group (3.6 ± 1.4 years) was longer than that of the palliative nonresective procedures group (1.5 ± 0.2) and the albendazole therapy-alone group (1.0 ± 0.4). The 5-year survival rates after palliative resection and liver transplantation were 40.0% and 66.7%, compared with only 7.7% and 9.1% after palliative nonresective procedures or albendazole therapy alone. Therefore, we concluded that aggressive treatment with a multimodality strategy could contribute to prolonged survival in patients with advanced HAE. Moreover, the prognosis of the patients who received albendazole therapy alone or palliative nonresective procedures is poor.