Castleman?s Disease is a rare lymphoproliferative disorder. In the literature, only 29 cases, associated with the neck presentation in children, have been reported. This is another case report regarding a 2-year old child who presented with a persistent cervical lymphadenopathy. Final pathology, after undergoing exploratory neck dissection and surgical excision, revealed Castleman?s Disease. This report, augmented with a literature review of all the 29 cases, compares the clinical course of this patient with the other cases. In conclusion, although Castleman?s Disease carries a favorable prognosis in children, surgical excision is recommended to confirm the diagnosis and to rule out other causes.