We present the case of a patient with the rare triad of Von Recklinghausen's disease associated with a somatostatinoma and a gastrointestinal stromal tumour (GIST). The patient had recurrent jaundice, the typical somatostatinoma syndrome, positive MR imaging but negative ⁶⁸Ga-DOTATOC PET scanning in a histopathology-proven somatostatinoma tumour.