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Abstract: Background: To demonstrate the effect of an inherited predisposition in familial Esophageal Squamous Cell Carcinoma (ESCC) as opposed to the sporadic cancer form. Methods: Differences in age of onset, prevalence rates of double primary ESCC, and post-operative survival rates between ESCC cases with (N = 476) and without (N = 1226)a family history of upper gastrointestinal cancer (FHUGIC, defined as having one or more first- or second-degree relatives with cancer of the esophagus or gastric cardia) were analyzed. Results: Overall, familial ESCC cases show a significantly earlier onset age (51.9 ± 8.2 versus 53.4 ± 8.0, Pt -test = .000), a significantly higher prevalence rate of double primary ESCC (2.73% Versus 1.22%, adjusted with TNM: X MH 2 = 4.029, P = .045), and a worse prognosis than the sporadic cases (P wald = .049). In subgroup analyses, the familial cases showed earlier onset and poor survival at most subgroups as opposed to the sporadic cases, and the difference was greater in early-stage rather than in late-stage groups (Pt-test for difference in onset age in Tis,1N0M0, T2,3N0M0, and T2,3,4N1M0 were .002, .006, and .081 respectively; and P wald for difference in survival in Tis,1N0M0, T2,3N0M0, and in T2,3,4N1M0 were .010, .180, and .520 respectively). Conclusion: These findings suggest the existence of familial as opposed to the sporadic ESCC. By the theory of “two-hit” origin of cancer, these findings also suggest that the “first hit”, a genetic predisposition, is inherited in familial ESCC. [Copyright &y& Elsevier]