A n 18-year-old female was referred to the gastroenterology clinic with a 3-month history of postprandial vomiting, colicky lower abdominal pain and 3 kg weight loss. Her bowels were regular with no bleeding or mucous discharge per rectum. She had no urinary or gynaecological symptoms and no significant past history. Her paternal grandfather had died of colon cancer, aged 60 years. She was pale but abdominal examination was unremarkable. Full blood count revealed iron-deficiency anaemia and, at upper GI endoscopy, multiple fundal hyperplastic polyps were identified. Severe duodenitis was present although a Helicobacter pylori urease test was negative. At colonoscopy, numerous polyps carpeted the colon suggesting familial adenomatous polyposis (FAP). Biopsies confirmed the presence of dysplastic adenomata and also revealed submucosal pigmentation suggestive of melanosis coli. Fundoscopy revealed congenital hypertrophy of retinal pigment epithelium (CHRPE) and genetic testing revealed a mutation in the APC gene on chromosome 5. Her parents and sister were screened and found to be free of the disease clinically and not to harbour the mutation.She was counselled with regards to a restorative proctocolectomy but, at operation, multiple enlarged pigmented lymph nodes > 2 cm in diameter were found in the small bowel mesentery. Node biopsy and frozen section showed no evidence of malignancy but the presence of dense pigmentation (Fig. 1)