Castleman disease (CD) is an uncommon benign condition of uncertain etiology that causes progressive lymph node enlargement. Although the mediastinum is their common location, they also occur in other areas of the body, usually where lymph nodes are normally found. In addition, CD (the hyaline-vascular type) of the parotid gland is very rare, especially in childhood. We report a new case of CD arising from the parotid gland in 9-year-old male child. The clinical course, histopathological findings, radiological findings, and management of CD are reviewed.