The aim of this study was to describe the clinicopathological characteristics and prognostic factors of carcinoma of ampulla Vateri. The medical records of 32 patients (24 men, 8 women) were evaluated. Median age was 59 years (range, 36-80 years). The performance status at the time of admission of (European Cooperative Oncology Group) 18 patients (56.3%) were ECOG-1; 8 patients (25.0%) were ECOG-2. Fifteen patients had early stage, 15 patients had locally advanced stage. Twenty-eight of 32 patients underwent curative surgery. Eleven, nine, and four patients had high-, moderate-, and low-grade histology, respectively. Fourteen patients received adjuvant treatment. Ten out of 14 patients were treated with chemotherapy. ECOG performance status (P = 0.06), stage (P = 0.05), perineural invasion (P = 0.01), tumor grade (P = 0.01), and treatment with chemotherapy, chemoradiotherapy, or only radiotherapy (P = 0.001) had a statistically significant impact on overall survival, whereas only tumor histopathology (P < 0.001) was shown to have a statistically significant effect on disease-free survival. Carcinoma of ampulla Vateri is a rare gastrointestinal tumor. Prospective trials with larger number of patients are needed to determine the prognostic factors to help select patients for adjuvant treatment.