CLINICAL HISTORYA10-year-old female child presented with history of o¡-and-on abdominal pain present for 2 years. The pain had become more frequent and colicky in the last 15 days. It was present in the hypogastrium and periumbilical area. There was history of constipation and an occasional episode of fresh blood with stools. She was the second of twins and the other twin sister was asymptomatic. On examination, melanosis was seen on the lips, oral mucosa, and tips of ¢ngers and toes since 1 year of age. Her mother had similar symptoms at 15 years of age for which she underwent an abdominal surgery. A part of the small bowel was resected and it was said to be studded with a few polyps. No written document or histopathology report is available. The mother has been asymptomatic ever since. The mother and the younger brother also had oral melanosis.The brother, however, hasbeen asymptomatic so far.On examination of the index case there was a mass present in the periumbilical area measuring 5 Â 3 cm. Reducible intussusception was present. Resection of the small intestine at two places was done with end-to-end anastomosis. Two segments (18 cm each) of the small intestine were resected, 9 inches from the duodenojejunal junction and at the jejunoilial junction. Both segments of the small intestine contained two polyps each. The largest polyp (5 cm in diameter) was seen 9 cm distal to the duodenojejunal junction, was pedunculated, and there were small cysts with mucoid material present at all levels of the wall including the serosa (Figure 1). The other three polyps were sessile and ranged from 2 to 4 cm in diameter. They too showed small cystic spaces with mucoid material.Microscopically the polyps revealed a classic morphology. Normal smallintestinal glands rested on a branching smooth muscle framework (Figure 2). There was no evidence of dysplasia. Transmural herniation of the mucosal glands admixed with smooth muscle ¢bers and lamina propria was seen until the serosa (Figure 3). Apart from this there were cystically dilated spaces (0.2^1 cm diameter)