We present the case of very rare periampullary gangliocytic paraganglioma in which we preferred proximal duodenopancreatectomy to local resection of the ampula of Vater because of not clear preoperative pathohistology and biologic behavior of the tumor. Although the definitive examination of the specimen proved its benign character we consider our procedure reasonable.

        Key words: mpullary gangliocytic paraganglioma – local resection – proximal duodenopancreatectomy