Purpose CDH1 mutation carriers have a 70% lifetime chance of developing gastric adenocarcinoma, therefore Prophylactic Total Gastrectomy (PTG) is recommended from age 20. PTG is associated with significant post-operative morbidity and may have life-long sequalae. This is the first systematic review on quality of life (QOL) following PTG. Methods A systematic review was conducted according to the PRISMA guidelines. The Cochrane Register, MEDLINE, Embase, CINAHL, PubMed, and Scopus were searched for relevant papers. The primary outcome was global QOL. Secondary outcomes included physical, psychological, social and financial effects. Results 1152 manuscripts were screened and 8 papers were included, including a total of 388 patients. Across all studies there was a decrease in QOL scores by up to 50% in the months following PTG, this tended to return to baseline after 1 year, 88-100% of patients would undergo PTG again. Anxiety about developing cancer decreased after surgery, but there were significant long-term QOL issues related to physical symptoms such as dumping, psychological issues with depression and body image, financial and employment issues. Often these long-term issues did not improve and some studies found worsening QOL at 2 years due to these sequalae. Conclusion PTG is the only intervention that can prevent gastric carcinoma in CDH1 mutation carriers. Surgery leads to a transient decrease in overall QOL. However, there are significant long-term physical, psychological and social issues. Despite this, patients are generally satisfied with their choice. This review can further assist in counselling these patients for this life-changing surgery.