Background: Pancreatic endocrine neoplasia (PEN) is an entity with an indolent course and prolonged survival. The aim of the current study was to review the prognostic factors and outcome of patients with PEN at 1 institution. Methods: Records of patients with the diagnosis of PEN in the period of 1980 to 2001 were reviewed. Descriptive statistics and log-rank test were used; significance was defined as P < .05. Results: Of 49 patients, 25 had functional and 24 nonfunctional tumors. Surgery was performed in 43 patients, 36 with curative intent. With a median follow-up of 31 months, there were 5 deaths. Symptoms were controlled with surgical resection in 86%. Median disease-free survival (DFS) was 46 months; actuarial 5-year disease-specific (DSS) was 72%. Factors associated with better DFS and DSS were absence of liver involvement (P = .02) and resection of the primary tumor (P = .04). Conclusions: Surgical resection and absence of liver metastases are associated with better DFS and DSS in patients with PEN. © 2007 Excerpta Medica Inc. All rights reserved.