The hereditary condition known as Peutz-Jeghers syndrome is characterized by mucosal pigmentation and gastrointestinal polyps. The polyps, usually pedunculated hamartomas, are significant only for the symptoms they cause. Intramural lesions also have been described with gross microscopic features that are often interpreted as malignant. Careful evaluation may show most of these lesions to be enteritis cystica profunda. These rarely diagnosed but benign tumors have very different implications for treatment and prognosis.