Islet cell carcinoma (ICC) of the pancreas is a rare, indolent malignancy associated with higher resectability rate and better survival than ductal carcinoma. This retrospective study presents results of surgical treatment from a single institution. From 1985 through 1993 a total of 64 patients (36 men, 28 women) were surgically treated for ICC. Ages ranged from 22 to 80 years (median 55 years) with a median postoperative follow-up of 39 months (range 10-97 months). Of the 64 patients, 30 (47%) had functioning and 34 (53%) nonfunctioning tumors. Gastrinoma (n = 11) followed by glucagonoma (n = 6) and insulinoma (n = 4) were the most common functioning tumors. In the patients undergoing a laboratory study, 67% of the nonfunctioning tumors had elevated peptide hormone levels. Potentially curative resections were performed in 17 patients (26%), palliative procedures in 35 (55%), and exploratory laparotomy alone in 12 (19%). One patient (2%) died within 30 days after operation. Symptomatic improvement was achieved in 96% of patients with a mean duration of 22 months. Three- and five-year survivals were 66% and 49%, respectively. In patients with curative resection, the disease-free survival at 3 years was 53% (95% CI: 32-86%). The presence of diffuse hepatic metastases was a predictor of poor survival at 3 years (74% versus 58%;p = 0.05); there was no statistically significant difference in survival between functioning and nonfunctioning groups (p > 0.1). Although curative resection for ICC is rare, meaningful palliation can be achieved in most patients with rare mortality and acceptable morbidity.