Objective To deeply explore the mechanism and treatment strategies of this rare UCD (unicentric Castleman disease,UCD), this paper reported the clinical data of 4 cases of UCD in children’s head and neck and reviewed relevant literature. Methods It was retrospectively analyzed for the clinical data of 4 children with UCD in the head and neck region admitted to Children’s Hospital Affiliated to Soochow University from September 2022 to March 2025. Comprehensive imaging examinations including B-ultrasound, CT and MRI of the head and neck masses were conducted before the patients were scheduled for surgical resection. Regular follow-up was conducted to observe the situation of wound healing and recurrence. Results All 4 children presented with painless masses in the head and neck region as the main symptom. There were no clinical manifestations such as repeated infection of the masses or nerve damage. The masses of all cases were successfully removed surgically. Postoperative pathology indicated lymphoid tissue and follicular hyperplasia. All 4 cases were diagnosed as Castleman disease (hyaline vascular type). Postoperative follow-up showed normal wound healing in all 4 children without any recurrence. Conclusions Clinically, UCD in the head and neck region of children is a rare lymphoid tissue hyperplasia disease. Its clinical manifestations are mainly painless mass hyperplasia in the head and neck region. Its imaging examinations have certain specificity. Pathological diagnosis is required for confirmation. Currently, surgical resection is the main treatment method for UCD. Some patients may require additional treatment.