Introduction: The authors present a case review of a localized Peutz-Jeghers syndrome of the caecum, emulating a carcinoma. Methods: The patient who presented with insignificant family history with a diagnosed stenosing tumor of his appendix and histological findings of highly suspected adenocarcinoma, was indicated for surgical revision and right-sided hemicolectomy. Results: The patient underwent laparoscopically assissted right-sided hemicolectomy and his postoperative course was adequate. The final histological finding diagnosed solitary hamartoma of the Peutz-Jeghers polyp. Conclusion: Differential diagnostic reasoning in patients with negative family history and unclear case history with findings of polypous tumorous lesions of the GIT, should also consider the Peutz-Jeghers syndrome. The syndrome is related to a high risk of malignancies in the elderly.

        Key words: Peutz-Jeghers syndrome – caecum – laparoscopic resection