Castleman disease, a rare lymphoproliferative disorder with diverse features, is often misdiagnosed due to rarity and similarity to malignancies.The study analyzed the clinicopathological characteristics, diagnostic challenges, and outcomes of head and neck Castleman disease (HNCD) to improve diagnostic accuracy and optimize management strategies.Retrospective study of 21 HNCD patients treated at Beijing Tongren Hospital (2007-2024). Demographics, presentation, imaging, histopathology, treatment, and outcomes were analyzed using t-tests, Mann-Whitney U, and Fisher's exact tests.Mean age 36.1; 52.4% female. Mainly painless cervical masses (95.2%). Histology: 85.7% hyaline vascular, 14.3% plasma cell. Imaging showing non-specific hypoechoic nodules (ultrasound) and homogeneous enhancement (CT/MRI). Surgical excision (85.7%) achieved 76.2% complete remission (mean follow-up: 85.2 months). Tumor size (34.6 ± 15.3 mm) didn't correlate with outcome (p = 0.756). One plasma cell patient (4.8%) died of unrelated causes. No recurrences or malignant transformations were observed.HNCDs are predominantly unicentric and can be cured with complete surgical resection. Due to the lack of specificity of imaging and laboratory findings, diagnosis relies on histopathology. Collaborations among multiple centers and molecular studies are essential to advancing the precision treatment of HNCD.