Patients with familial adenomatous polyposis (FAP) are at increased risk of developing duodenal and jejunal adenocarcinomas. The aim of this study was to assess the usefulness of double-balloon enteroscopy- (DBE-) assisted chromoendoscopy for the detection and characterization of small-bowel polyps in patients with FAP. We performed a prospective evaluation of patients with clinically and genetically proved FAP who were enrolled in an endoscopic surveillance program. DBE was performed using a Fujinon intestinoscope (FN 450P 5/20; Fujinon Corp., Omiya, Japan), and chromoendoscopy was performed using indigo carmine. The severity of small bowel polyposis was based on the Spigelman-Saurin classification. Nine patients underwent DBE-assisted chromoendoscopy. Small-bowel polyps (including papillary adenomas) were detected in seven patients (88 %). The mean depth of small-bowel insertion was 180 cm (range 120-320 cm). The mean Spigelman-Saurin score was 4.6 (range 0-8). Jejunal polyps were detected in six patients (67 %). Chromoendoscopy aided in the detection of additional polyps in two patients. In one patient the polyps were flat and only visible with chromoendoscopy (biopsy confirmed these to be adenomas). Jejunal polyps and advanced neoplasms were more frequent in patients with APC gene mutations in exon 15. The following endoscopic therapies were performed: polypectomy (n = 1), duodenal mucosectomy (n = 1), and ablation therapy with argon plasma coagulation (n = 2). DBE was found to be a helpful method for the evaluation of small-bowel polyps in patients with FAP. DBE-assisted chromoendoscopy was of further assistance for the detection of jejunal polyps.