Long-term follow-up of children with the Zollinger-Ellison syndrome (gastrinomas) suggests that surgical management is still advantageous. Twenty-eight children with the Zollinger-Ellison syndrome have been followed up to 21 years after their initial surgical procedure. Six of seven children with less than total gastrectomy, all of whom underwent operation before the introduction of histamine H2-receptor antagonists, are known dead from complications of continued gastric hypersecretion and tumor growth. Sixteen children had a total gastrectomy, with no operative deaths, and only one died of progressive tumor growth, even though 14 had evidence of metastatic islet-cell carcinoma. Follow-up serum gastrin measurements have been obtained for 13 patients with total gastrectomy, and 5 patients now have a normal serum gastrin levels. Malignant gastrinomas in children have been slow growing, indolent, and compatible with long life. The biologic behavior of malignant gastrinomas appears to be more favorable in the young patient. Total gastrectomy can be done safely in children with the Zollinger-Ellison syndrome and effectively controls gastric hypersecretion when all gastrin-producing tumor cannot be excised. Surgical exploration and an attempt at "curative" tumor excision, even when tumor is extrapancreatic and in lymph nodes, appear worthwhile in selected patients.