Multiple endocrine neoplasia type 1 (MEN1) is inherited in an autosomal dominant 
fashion and predisposes to the development of hyperplastic or neoplastic changes 
in the parathyroid and pituitary glands and the endocrine pancreas, along with 
numerous other characteristic tumors and features. The management of each entity 
differs to some degree from their sporadic counterparts, while the lack of a 
genotype-phenotype correlation requires lifelong clinical, biochemical and 
radiological screening for the development of new tumors. While the syndrome 
itself is relatively rare (a prevalence of 1-10/100 000), it is likely that 
health-care practitioners from numerous specialities will occasionally encounter 
a patient with MEN1 and therefore a basic knowledge of the syndrome is 
important. In addition, many of the associated tumors are seen commonly in 
sporadic form, and a judicious policy is therefore required in deciding how 
thoroughly patients who develop these tumors should be screened for MEN1. The 
current literature on MEN1 is reviewed and key learning points are suggested for 
the clinician.