Inactivating germline variants in the CDH1 tumor suppressor gene cause the hereditary diffuse gastric cancer syndrome. Total gastrectomy is recommended for prevention, although it is associated with adverse outcomes and chronic health risks. Gastric cancer surveillance is an alternative to surgery; however, upper gastrointestinal endoscopy is limited by poor sensitivity. Cancer surveillance requires accurate detection of early carcinoma and patient-specific disease penetrance estimates. Current clinical care should incorporate up-to-date information on variable disease penetrance, which does not seem to correlate with CDH1 genotype. Affected patients and families warrant a balanced presentation of options for cancer surveillance and prophylaxis.