Background Gene expression analyses indicate that there are 152 genes of which the expression differs significantly in esophageal squamous cell carcinoma (ESCC) cases with positive as opposed to those with negative family history of upper gastrointestinal cancer (FHUGIC) in the high-incidence area for ESCC in northern China. However, the question as to whether there is any difference of onset age or survival rates in the familial and sporadic cases of ESCC in the area is unknown. Aims To investigate the differences of onset age or survival rates in the familial and sporadic cases of ESCC for surgically treated ESCC patients from the high-incidence area. Methods Retrospective analyses were performed on the clinicopathologic and survival data of ESCC cases (N = 1715) who had undergone surgery alone from 1985 to 1994 in Hebei Cancer Center, a provincial cancer center established primarily to treat esophageal cancer in the high-incidence area, to investigate the differences. All the patients had been native residents of the high-incidence area in northern China. Student's t-test was used to test the difference of onset ages, and Cox Proportional Hazard Model was used to examine the differences of survival rates in the familial and sporadic cases of ESCC. Results Although the familial cases of ESCC had had a significantly earlier onset than the sporadic cases (P < 0.00), they experienced relatively lower survival rates than the sporadic cases after surgery. The differences of survival rates in the familial and sporadic cases were significant for patients above the age of 50 years (P-Wald = 0.04) and for the T-is,T- 1 N0M0 group (P-Wald = 0.04), the differences were bigger for early-staged than for the later-stage groups, and the differences persisted when adjusted for or stratified by confounding factors such as sex, age (under versus above the age of 50 years), smoking, drinking, cancer segment location, surgery year (calendar year), stage (UICC 4th Ed, 1987), and Resection category. Overall, cases under the age of 50 years old showed a higher survival curve than cases above the age of 50 years old, and this was especially true for the familial case group where the difference was significant (P-Wald = 0.03). Conclusion The findings suggest that the familial ESCC may develop earlier, and may have a poorer prognosis than the sporadic ESCC. Both earlier onset and poorer outcome may be important features for the familial as opposed to the sporadic cases of ESCC. The association between younger onset age and higher survival rates found for the familial cases may indicate some survival benefit for early discovery for people with positive FHUGIC in the high-incidence area.