Multiple endocrine neoplasia (MEN) type 1 exhibits an autosomal dominant pattern of inheritance and results from mutation of the MEN-1 gene that is a tumor suppressor gene acting on the transcriptional level. The disease is characterized by a variety of neuroendocrine neoplasias and hormone excess syndromes. The major components of MEN-I are hyperparathyroidism due to multiple parathyroid adenomas, pancreatic or duodenal neuroendocrine tumors, and pituitary adenomas, in addition to some less common neoplastic manifestations. The development of the tumors often follows a substantially similar pattern: the initial lesion is a diffuse hyperplastic proliferation of the affected endocrine tissue with bilateral involvement of pair organs, followed by development of multiple micro- and, eventually, macronodular lesions. Its unpredictable course causes that there is controversy regarding treatment of the different manifestations and screening modalities of this disorder. This article reviews our current approach to the diagnosis, surveillance and management of these patients.