Neuroendocrine tumours (NETs) of the gastrointestinal tract (GI) and pancreas are a rare and heterogeneous group of neoplasms with unique tumour biology, natural history and clinical management issues.1–4 Most NETs are sporadic, but they can be part of familial cancer syndromes such as multiple endocrine neoplasia type 1 (MEN1), neurofibromatosis type 1 (NF1), tuberous sclerosis (TS) or Von Hippel-Lindau (VHL) syndrome.5–9. The gene discussed is NF1; the disease is neoplasm.