Although advanced hepatocellular carcinoma (HCC) with extrahepatic metastasis is recommended to be treated by a systemic chemotherapeutic agent without local treatment targeting the liver, studies reported that causes of death in these patients were mostly from progression of intrahepatic lesions. Thus, this study investigated prognosis and factors predicting survival in these patients so as to evaluate the role of local treatments against intrahepatic lesions when the patients already had extrahepatic metastasis. This retrospective study evaluated medical records of 277 patients with HCC and extrahepatic metastasis. The median survival was 5.9 months, and 257 patients died during the follow up. Factors affecting survival of HCC patients with extrahepatic metastasis were poor response to treatment of hepatic lesions (HR 2.207; 95 % CI; p < 0.001), applying local treatment specifically targeting intrahepatic lesions (HR 0.591; 95 % CI 0.436–0.803; p = 0.001), intrahepatic tumor size larger than 3 cm (HR 2.065; 95 % CI 1.444–2.954; p < 0.001), and ECOG performance status 2 or higher (HR 1.543; 95 % CI 1.057–2.253; p = 0.025). The patients with either complete or partial response to the therapy had 1- and 2-year survival rate of 48.8 and 12.1 % whereas patient with either stable or progressive disease had 1-year survival rate of 11.4 %. These results suggest that even in the HCC patients with extrahepatic metastasis, effective local treatment may still be beneficial for the survival especially in patients with acceptable performance status.