DOI: 10.36347/sjmcr.2022.v10i07.024

Pages: 697-699

Peutz-Jeghers syndrome (PJS) is a rare hamartomatous polyposis of the gastrointestinal GI) tract, sometimes associated with pigmentation around lips and oral mucosa. The case of a 4-year-old girl who presented with duodenal obstruction is reported. Along with multiple small polyps in the duodenum, a large polyp was found to be the cause of the duodenal obstruction. Histologically most of the polyps were hamartoma. PJS is a rare autosomal dominant disease. Most patients have recurrent episodes of polyposis in the gastrointestinal tract causing gastrointestinal obstruction as well as these patients have an increased risk of both gastrointestinal and non-gastrointestinal malignancy and need regular screening.