Peutz-Jeghers syndrome (PJS) is an autosomal dominant condition characterized by the association of gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition McGarrity, Amos, Baker (Peutz-Jeghers Syndrome, GeneReviews(R), National Center of Biotechnology Information.). Intussusception and malignant polyps are not rare complications of PJS, where the lifetime risk of intussusception is 48% and the possibility of developing any cancer by age 65 years is 37% Kopacova, Tacheci, Rejchrt, Bures (Peutz-Jeghers syndrome: diagnostic and therapeutic approach. World J Gastroenterol 2009;15:5397-408.). Very few cases of malignant polyps causing intussusception associated with synchronous malignant polyp in PJS have been reported to date Cai, Tian, Zhou, He, Hu, Deng (Jejunal intussusception and polyps with different types of malignant transformation in Peutz-Jeghers syndrome: report of a case. Oncol Lett 2013;5:239-41.). We describe a case of a patient with PJS presenting with symptomatic jejunal intussusception and an incidentally found malignant hamartoma in the sigmoid colon.