The Peutz-Jeghers syndrome is a rare dominant autosomic, entity characterized by hyperpigmented lesions on the lips, hands and feet; with presence of gastrointestinal polyps producing acute or chronic anemia, intestinal obstruction, and/or abdominal pain. This polyps histologically are hamartomas; recent studies indicate a real risk for transformations in the malignant neoplasia. The high and low endoscopies and the intraoperative enteroscopy with polypectomy are the election treatment, improving prognosis quality on these patients. We describe a familiar case of a female patient 24 years old showing a repeated picture of intestinal subocclusion; her brother presented a similar clinical picture, and her mother presented the same syndrome, dying of carcinoma in the colon; also her child, at one and a half year old presented hyperpigmented lesions on the lips.