Hereditary diffuse gastric cancer was discovered 25 years ago. During the last 25 years, the diagnostic standards and molecular mechanism of this type of hereditary gastric cancer have been established. With a better understanding of this disease, germline CDH1 mutation has been linked to hereditary diffuse gastric cancer with autosomal-dominant inheritance pattern. Therefore, strategies on genetic screening and follow-up have been evolved for CDH1 mutation carriers. In China, the studies on hereditary diffuse gastric cancer are sparse. We hope this article will bring more awareness and attention of this peculiar disease to Chinese physicians.