The article reviews our experience about tumours of the papilla of Vater. 30 patients were operated on between 1996-1999. The mean age was 58 years, the leading symptom (73%) was jaundice. In 23 patients preoperative ERCP was successful and was completed in 15 cases with EST, in 8 cases with biliary drainage. In 3 patients--despite benign endoscopic histology--intraoperative frozen section, and in one patient final histology confirmed malignancy. Altogether in 27 patients was malignant transformation of the papilla observed. In 15 cases pylorus-preserving pancreatoduodenectomy (PPPD), in 2 cases local excision (LE), while in 10 patients palliative procedures were performed. Resection rate (PPPD + LE) was 63%, while radical resection (PPPD) was performed in 55.5% of all cases. During 26 months mean follow-up survival rate was altogether 74%, and 80% in radically resected patients. We review the literature, describe the difficulty of histological and describe our experience and practice in the treatment of this rare tumour.