Abstract: With the development of the molecular genetics, we have got a better understanding about the fundamentals of genetics, pathogenesis, and clinicopathological characteristics of the familial gastric cancer. Genetic tests should be carried out in familial gastric cancer patients and reasonable preventive intervention could effectively improve the prognosis of patients with familial gastric cancer who have CDH1 germline mutations. This article reviews the research progress on familial gastric cancer at home and abroad in recent years.