AIM: To clarify the therapeutic strategies and prognosis factors of primary clear cell carcinoma of the liver (PCCCL).METHODS: The clinical pathological data of 64 patients with PCCCL treated with hepatectomy in our hospital from January 2000 to January 2006 were analyzed retrospectively.The patients were divided into two groups to make treatment analysis: curative resection only ( n = 40); and curative resection and postoperative chemotherapy with calcium folinate and tegafur ( n = 24).Meanwhile, the PCCCL patients were subdivided into two subgroups on the basis of the proportion of clear cells in the tumor for pathological analysis.There were 36 cases in subgroup A for which the proportion of clear cells was more than 70%, and 28 cases in subgroup B for which the proportion was less or equal to 70%, comparing analysis of median survival time of the counterpart groups.Univariate and multivariate analyses were performed to examine factors that affected clinical prognosis, recurrence and metastasis.RESULTS: Median survival period of the curative surgery group was 38 mo, while the counterpart was 41 mo.Median survival period for group A was 41 mo, while group B was 19 mo.The Kaplan-Meier method showed that capsule formation, preoperative liver function, hepatitis C virus infection, large vascular invasion and multiple tumor occurrences were related to disease-free survival.Cox regression analysis showed that the clear cell ratio, capsule formation, preoperative liver function and large vascular invasion were independent risk factors for overall survival.CONCLUSION: Postoperative chemotherapy has no obvious effect on survival of patients with PCCCL.Clear cell ratio, capsule formation, preoperative liver function, and vascular invasion were independent risk factors for prognosis.