NF-1-associated inflammatory polyps probably represent specific GI manifestations of this disorder, and should be considered, particularly in patients with GI symptoms. They should be distinguished from inflammatory fibroid polyps and from juvenile-like changes associated with ganglioneuroma/ganglioneuromatosis and neurofibroma/neurofibromatosis. Their aetiology remains obscure, but different mechanisms, including NF-1 inactivation, NF-1-associated vasculopathy, and localised mucosal prolapse/damage caused by motility disorders, might be involved.