Conclusion:
Although PJ polyps are unusual in children, they should be considered as a potential pathological lead points in case of recurrent jejunal intussusceptions, especially in presence of characteristic dark muco-cutaneous macula, or familial history. Besides the risk of serious gastro-intestinal polyp-related complications, PJS is associated to an increased risk of cancers. Early identification of this condition is then important for symptomatic or at risk patients, as it permits to establish a strict and regular systemic surveillance program, which can considerably improve quality of life and prognosis of these individuals.