Abstract
Multiple endocrine neoplasia type 2 (MEN2) is a rare autosomal dominant disorder that predisposes patients to medullary thyroid cancer (MTC), pheochromocytoma (PHEO), and primary parathyroid hyperplasia (PHPT). MEN2 is subclassified into three distinct syndromes: Multiple endocrine neoplasia type 2A (MEN2A), multiple endocrine neoplasia type 2B (MEN2B), and familial medullary thyroid cancer (FMTC). All three syndromes are the result of mutations of the RET proto-oncogene. The diagnosis and management of MEN2 syndromes in children is discussed.