Juvenile polyposis syndrome (JPS), recognized in 1964 for the first time (McColl 1964), is considered a precancerous condition that can predispose individuals to gastrointestinal cancers. JPS is characterized by gastrointestinal (GI) manifestations, predominantly multiple hamartomatous juvenile polyps (Dal Buono et al. 2022; Shen et al. 2020). The term “juvenile” reflects the histological subtype of polyp distinguished by stromal edema, cystic dilation of glandular ducts, and mucosal infiltration by inflammatory cells and should not be misinterpreted by clinicians. At the time of diagnosis, the average age of the patients is 18.5 years (Matsumoto et al. 2023). The colon/rectum (98%) polyp was reported to be the most frequent GI polyp in JPS, followed by the stomach (14%), duodenum (7%), and jejunum and ileum (7%). Proximal colon polyps account for 70% of colonic polyps (Dal Buono et al. 2022). Individuals carrying mutations in SMAD4 are more likely to develop gastric lesions and heredity hemorrhagic telangiectasia (HHT), defined by mucocutaneous telangiectases as well as arteriovenous malformations in the brain, liver, and lungs (Matsumoto et al. 2023). Symptoms of HHT may develop in childhood (Cohen et al. 2019). Upon reaching the age of 60, those suffering from JPS are at a 68% risk of contracting colorectal tumors. Additionally, they pose a cumulative risk of 21% for upper GI cancers, such as gastric, duodenal, and pancreatic cancer. It is estimated that approximately less than 1 person out of every 100,000 suffers from JPS (Dal Buono et al. 2022).