A 46-year-old man developed a peptic ulcer disease and a diagnosis of Zollinger-Ellison Syndrome was strongly suspected following gastric acid studies. Plasma gastrin level was found to be elevated. The operation revealed a pancreatic tumor localized at the pancreas tail. The resected tumor tissue was proved to contain large amounts of immunoreactive gastrin. In addition, calcitonin, VIP and insulin were found to be present either as a result of immunofluorescence studies or the respective radioimmunoassays. Therefore, this was a case of a multiple hormone producing tumor of pancreas islet cell origin, and the symptoms due to gastrin hypersecretion were the main clinical features. In addition, 48 cases of Zollinger-Ellison Syndrome reported in Japanese literatures were reviewed.