Context: Multiple endocrine neoplasia 1 (MEN 1) is an autosomal dominant disease presenting as hyperplasia and neoplasia of parathyroid, pituitary and enteropancreatic tissues. Over 90% of gene carriers develop phenotypic disease by age 30 years, potentially with onset of asymptomatic disease during childhood and adolescence. Objective: To describe the paediatric and young adult manifestations of MEN 1. Design: Descriptive retrospective study of 180 patients with a common MEN1 genotype. The paediatric and young adult (age