Multiple endocrine neoplasia 1 (MEN1) is a hereditary syndrome associated with a number of endocrine and nonendocrine tumors. Unfortunately, a diagnosis of MEN1 often is delayed until after the patient has developed an advanced or second MEN1-related tumor. Nurses need to be familiar with hallmark signs of this syndrome to facilitate an early diagnosis and appropriately refer families for genetic assessment and, ultimately, develop a long-term plan for early detection and intervention for all family members at risk for MEN1.