Patients with multiple endocrine neoplasia type 1 (MEN-1) are an unusual challenge to the endocrine surgeon. Pituitary disease is often treated without surgery, but nearly all patients will require parathyroidectomy for parathyroid hyperplasia. Subtotal parathyroidectomy can be accomplished with a very low rate of permanent hypoparathyroidism and an acceptable rate of recurrent hyperparathyroidism. The treatment of pancreaticoduodenal disease is quite controversial. Even when associated with the Zollinger-Ellison syndrome, early and aggressive surgical treatment should be considered to influence the hormonal syndrome as well as to address the malignant potential of both pancreatic and duodenal tumours. This includes distal pancreatectomy, enucleation of pancreatic head lesions, and duodenotomy with the resection of gastrinomas. Many patients may be completely cured of the manifestations of their disease. As MEN-1 is an uncommon entity, there are very few prospective, randomized data upon which to base surgical judgements.