The aim of this report is to present a case of unicentric hyaline vascular Castleman disease in the neck and to discuss its treatment in the light of the current literature. A 52-year-old man presented with a painless, non-progressive swelling in the right submandibular region for one year. Physical examination revealed a 2 × 3 cm firm, fixed mass located outside the submandibular gland. Radiological tests identified a well-circumscribed lesion located outside the submandibular gland. As lymphoid cells were reported in fine-needle aspiration cytology, an excisional biopsy was performed and hyaline vascular Castleman disease was diagnosed. As the disease was unicentric, no further treatment was planned, and the patient was taken into follow-up. During five years of clinical and radiological follow-up, there was no evidence of recurrence.