Clinicopathologic findings and clinical follow-up data of 31 patients with non-icteric ampullary carcinoma (NIAC) and 111 patients with icteric ampullary carcinoma (IAC) were retrospectively compared. All of the IAC patients presented with obstructive jaundice. Twenty-three of the 31 NIAC patients developed abdominal pain and/or fever caused by cholangitis or pancreatitis, and the remaining eight patients were asymptomatic. The two groups were not significantly different in age, sex, size of the tumor, macroscopic type, lymph node metastasis, perineural invasion, lymphatic permeation, and venous invasion. Eighteen of the 31 NIACs (58%) were in stages I and II, whereas 25 of the 111 IACs (22%) were in stages I and II (p less than 0.01). Seventeen of the 31 NIACs (55%) were papillary adenocarcinoma, compared with 39 of 111 IACs (35%) (p less than 0.05). As to involvement of the biliary tract, the NIAC showed an intraluminal papillary growth in 14 cases (45%), whereas the IAC showed a periductal invasion in 58 cases (52%) (p less than 0.05). The cumulative 5-yr and 10-yr survival rates of 31 patients with NIAC were 57% and 57%, compared with 32% and 23% of 105 patients with IAC (p less than 0.05; p less than 0.01). The survival curve of the NIAC was significantly better than that of the IAC (p less than 0.01). Non-icteric presentation had no independent prognostic value, as determined by multivariate regression analysis. The NIAC fares better than the IAC, because the NIAC includes a greater number of early ampullary carcinoma and papillary adenocarcinoma. The detection of NIAC may therefore product an improvement in the clinical course of ampullary carcinoma.