Jaundice is a common manifestation of ampullary carcinoma. The aim of this study was to evaluate the correlation between jaundice at initial presentation and the degree of tumor spread and to determine the prognostic significance of jaundice in patients with ampullary carcinoma. Fifty-nine patients who had undergone curative resection for ampullary carcinoma were analyzed retrospectively. Jaundice was defined as a total bilirubin serum concentration of > or = 3 mg/dl. The median follow-up time was 106 months. Jaundice was noted at the time of initial presentation in 43 (73%) patients. Jaundice at presentation correlated with lymph node metastasis (p < 0.0001), lymphatic vessel invasion (p < 0.0001), invasion into the pancreas (p = 0.0007), and vascular invasion (p = 0.0487). Pancreatic invasion was absent in patients without jaundice. Superior mesenteric nodal involvement was more frequent in patients with jaundice (15/43) than in those without (0/16) (p = 0.0062). The survival of patients with jaundice (median survival 48 months; cumulative 10-year survival rate 39%) was worse than for patients without jaundice (median survival time not available; cumulative 10-year survival rate 86%) (p = 0.0014). In conclusion, jaundice at presentation predicts advanced-stage ampullary carcinoma and a poor prognosis. Pancreatic invasion and superior mesenteric nodal involvement were not observed in nonjaundiced patients.