Castleman disease is a rare and complicated disease. A case of Castleman disease (CD) in a 40-year-old woman on the neck was reported and literature review was made to understand the occurrence, clinical characteristics, diagnosis and differential diagnosis, treatment, and prognosis of Castleman disease. Because of the rarity of CD, its occurrence in the general population and the typical clinical manifestations are still lacked. There are two types of CD, namely localized CD and multicentric CD, both of them are not easy to be diagnosised. CT scans in preoperative is helpful for the clinical diagnosis, while histopathological examination and lymph node biopsy are beneficial for the early diagnosis. The treatment result is based on the histopathological examination and lymph node biopsy. The prognosis is different with the type of CD. For the localized CD, the prognosis is usually good, while for the multicentric CD is bad. Both of localized CD and multicentric CD have a certain reoccurrence rate.