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Introduction: Familial adenomatous polyposis (FAP) is a rare disease with hundreds to thousands adenomatous polyps in large intestine which inherits in the form of autosomal dominant, but 25% of patients have no positive family history. Rectorrhagia and rectal prolapsus are the most prevalent symptoms. Diagnosis is based on clinical findings and results of colon investigation, and prompt colectomy prevents carcinoma in patient. In this article a case report of familial adenomatous polyposis is presented. Case: The patient is a 17 years old teenager, a known case of familial adenomatous polyposis, who was refered with severe obstruction signs of gastrointestinal tract. The disease was diagnosied when he was 5 years old. In 6 years old he was treated by partital colectomy and in 11 years old by total colectomy and ileorectal anastomosis. The patient was investigated by endoscopy and small bowel transit. Because of many polyps in stomach, duodenum and jejunum polypectomy, gastrectomy and duodenotomy were done. Diffuse carcinoma in situ was reported in pathology. Conclusion: Rectal and colonic polyps are rarely obsereved below 10 years old, but rectorrhagia and rectal prolapsus suspects the diagnosis of FAP in children. In the case of dysplasia and carcinoma abdominal pain, anemia and weight loss will be presented. In children with suspecting FAP, screening of extracolonic manifestation and dysplasia should be considerd and elective surgery should be done.

Keywords: Familial adenomatous polyposis, rectorrhagia, cell, dysplasia, gastrointestinal tract.

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