OBJECTIVES: Castleman's disease is an uncommon disease of benign lymph node hyperplasia primarily affecting the mediastinum, with the head and neck region being the second most common site.CASE REPORT: A 27-year-old woman was admitted to our department due to a left lateral cervical mass. After a complete clinical and imaging examination, a neck dissection was performed, in which a mass, size 3.5 x 2.7 x 4.5 cm, was excised. The patient was diagnosed with Castleman's disease of the neck after histopathological examination.CONCLUSIONS: The diagnosis of Castleman's disease is always a clinical challenge, as the patient commonly presents with nonspecific signs and symptoms resembling other lymphatic diseases. Consequently, Castleman's disease should be in the differential diagnosis of congenital, inflammatory, or neoplastic cervical alterations. A review of literature, including histopathological characteristics, differential diagnosis, and treatment options is also presented.