Twenty-five per cent of patients with neurofibromatosis type 1 (NF1) have gastrointestinal involvement and malabsorption symptoms have been reported. We describe, for the first time, a patient with NF1, in whom gastrointestinal symptoms were due to coeliac disease (CD). Although this could be a coincidental association, we suggest that CD should be taken into account in the differential diagnosis of diarrhoea occurring in NF1 patients.