A case of Zollinger-Ellison syndrome with some peculiar aspects is described. The disease started with painless dyspepsia, watery diarrhea, relevant loss of body weight and absence of peptic ulcer. After a steroid treatment performed at home without any rationale, diarrhea stopped totally for 18 months and body weight returned to normal. Subsequently, the disease recurred with more typical clinical characters: juxtapyloric ulcer, increase in serum gastrin levels and positive secretin test, presence of gastrinomas at angiography. The monitoring of gastric pH showed a persistent hyperacidity during medical treatment with H2 and muscarinic antagonists. The localization of gastrinomas was extrapancreatic, at the root of jejunal mesentery. Excellent results were obtained by surgical treatment which consisted in resection of the principal neoplastic masses with total gastrectomy. The patient's general conditions after two years are good.