multiple endocrine neoplasia type 2B (MEN2B) is an autosomal dominant genetic disease manifesting as medullary thyroid carcinoma, pheochromocytoma, multiple mucosal neuroma and Marfanoid habitus. It is clinically rare and severe[1]。 Thyroid involvement is often manifested as medullary thyroid cancer, which is prone to lymph node metastasis and has a poor prognosis. Multiple neuroma of oral mucosa is an early clinical manifestation of MEN2B. If stomatologists have some understanding of this disease, they can consider this disease when finding multiple neuroma of oral mucosa. It is recommended that patients conduct timely examination of systemic related diseases, which is conducive to early detection of multiple endocrine neoplasia, early diagnosis and improvement of prognosis. A case of MEN2B diagnosed and treated in the Department of Mucosa, School of Stomatology, Capital Medical University is reported.