We present a case of an adult male patient with multiple tumours, including colonic polyposis, colon adenocarcinoma, bilateral renal cysts, pancreatic lipomas, multiple abdominal nodules, a CNS mass and an atypical CML, harboring two germinal variants in CHEK2 and APC, respectively, associated with hereditary cancer predisposition. This evidence concerns the gene APC and chronic myelogenous leukemia, BCR-ABL1 positive.