Lynch syndrome (LS) is the most common hereditary syndrome that predisposes patients to colorectal cancer, and it accounts for 2-5% of the total burden of colorectal cancer. We report a case of a 61-year-old female affected by Lynch syndrome who underwent multiple adenocarcinoma resections, studied by F18-FDG-PET/CT for 5 years. This case report suggests a potential role of F18-FDG-PET/CT in the evaluation of patients affected by Lynch syndrome.