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 cystic kidney disease.