Introduction: Peutz–Jeghers syndrome (PJS) is a rare autosomal dominant syndrome associated with mucocutaneous pigmentation and hamartomatous gastrointestinal polyps. It is associated with increased risk of intestinal and extraintestinal malignancies. Case Report: This is a rare case of PJS with multiple polyps with features of villous adenoma and high grade dysplasia. A 48-year-old female presented with the complaints of bleeding per rectum since five months. Hyperpigmented spots were present on the buccal mucosa. On endoscopy, multiple polyps in distal colon, stomach, duodenum, and periampullary region were noted. Polypectomy was done and sent for histopathological evaluation. Grossly, the largest polyp was in sigmoid colon measuring 2.6 × 1.5 × 1 cm and smallest polyp was in stomach measuring 3 × 2 × 1 mm. On microscopy, polyps from transverse colon showed polypoidal tissue traversed by branching muscularis mucosae forming arborizing pattern. Polyps from sigmoid colon showed similar features with a focus of dysplasia and villous adenoma. Conclusion: Further the patient was advised to undergo mandatory periodic evaluation on follow-up.