Multiple endocrine neoplasia 2 (MEN2) is a hereditary syndrome associated with medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. Unfortunately, a diagnosis of MEN2 often is delayed until after the patient has developed an advanced MEN2-related tumor. Nurses should 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 developing MEN2.