Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papilla of Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 cases described in the literature to date, with gastrointestinal bleeding being more common. Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more than casual, being the frequency in normal population 1:3,000. We report an additional case of this association, presenting for the first time with simultaneous obstruction of the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/or NF-1 are reviewed, and updating of the histogenesis presented.