We describe herein a 72-year-old woman with tumor recurrence in the residual pancreas and metastasis to the liver following a pylorus-preserving pancreatoduodenectomy for multiple endocrine tumors in the head of the pancreas. Abdominal ultrasonography performed 7 years after the initial surgery detected new lesions in the residual pancreas and liver. After recurrence of endocrine tumors of the pancreas and metastasis to the liver were diagnosed, the lesions were successfully resected by total pancreatectomy with distal gastrectomy and both lateral segmentectomy and partial resection of segment 8. Genetic analysis using a blood specimen showed that this patient carried the multiple endocrine neoplasia type 1 (MEN1) gene mutation. One year after the second resection, the patient remains in good health using insulin and has not shown any sign of recurrence. This case report describes successful surgical resection for recurrence and metastasis of malignant endocrine tumors in a patient with the MEN1 gene mutation.