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Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by typical pigmented perioral maculesn pigmented spots in the oral mucosa and digits along with hamartomatous polyps in the gastrointestinal tract. The pigmented macules usually appear during infancy or early childhood and have a tendency to increase in size during adolescence. The characteristic clinical course includes recurrent episodes of polyp-induced bowel obstruction, abdominal pain and gastrointestinal bleeding. In addition to polyposis, the risk of gastrointestinal and extragastrointestinal malignancies is significantly higher in patients with PJS. We report two unique cases of Peutz-Jeghers syndrome in sibling with intestinal polyposis and mucocutaneous pigmentation with a definite family history. An overview on differential diagnosis and therapeutic aspects of the disease has been discussed.