APC and cancer: Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease caused by pathogenic variants in the adenomatous polyposis coli (APC) gene1 with reported incidence of one in 8,000 to 12,000 live births.2 The main hallmark of the disease is the presence of multiple colorectal adenomas, leading to a 100% lifetime risk of developing cancer if the colon remains in situ.3 To prevent the development of cancer, prophylactic colectomy or proctocolectomy is performed when the adenoma burden cannot be managed endoscopically or at the age of 18–25 years old.