Objective: This study was performed to investigate the clinical and pathological manifestations of Castleman disease in head and neck, and to summarize its clinical characteristics, experiences of diagnosis and treatment, and prognosis of patients. STUDY DESIGN: Retrospective study. SETTING: Hunan Provincial People’s Hospital. Methods: We retrospectively analyze the clinical data of 8 patients suffering from Castleman disease in head and neck who received treatment in Hunan people’s Hospital between 2013.1 to 2020.07, and review the relevant literature. Results: 7 of the 8 patients were characterized by single or multiple painless masses in head and neck. They were categorized as unicentric Castleman disease (UCD). Case 8 was clinically classified as multicentric Castleman’s disease (MCD) because the neck mass of this patient was found with discomfort such as anemia, fatigue and fever. The pathological types were hyaline-vascular type (HVT) in 6 patients, mixed type in 1 patient and the plasma cell type (PCT) in 1 patient. All the patients were operated to remove the mass and showed no recurrence in the following up for 4-93 months. Conclusions: The diagnosis of Castleman disease in head and neck should be confirmed by pathological examination. UCD is the main type in the clinical classification, and HVT is the main type in pathological. Surgical resection of the mass can be an effective treatment, and proper medical therapy should be supplemented to those with postoperative recurrence. The patients with UCD generally have a good prognosis. [KEY WORDS] Head and neck; Castleman disease; Diagnosis; Treatment