Vol. 8, Issue 4, Part A (2025)

Background: Castleman’s disease is a rare lymphoproliferative disorder that predominantly affects mediastinal lymph nodes but can involve any nodal region, including the head and neck. Involvement of the parotid gland is extremely uncommon and often mimics primary salivary gland tumors, making accurate diagnosis challenging. Case Presentation: We report a case of a 14-year-old male who presented with a gradually progressive, painless swelling in the right parotid region for one year. There was no history of trauma or systemic symptoms. Clinical examination revealed a solitary, firm, mobile mass without overlying skin changes. Routine laboratory investigations were unremarkable. Ultrasonography demonstrated a hypoechoic lesion within the right parotid gland, suggestive of pleomorphic adenoma. Initial and repeat fine needle aspiration cytology yielded reactive lymphadenitis. Due to persistence of the swelling, surgical excision was performed. Histopathological examination revealed partial effacement of nodal architecture, atretic germinal centres traversed by hyalinised vessels (“lollipop appearance”), concentric layering of mantle zone lymphocytes (“onion-skinning”), perivascular hyalinisation, and extensive interfollicular vascular proliferation, confirming the diagnosis of hyaline vascular type unicentric Castleman’s disease. Conclusion: Castleman’s disease involving the parotid gland is a rare clinical entity that should be considered in the differential diagnosis of persistent parotid swellings in children and adolescents, particularly when imaging suggests a benign neoplasm and cytology remains inconclusive. Histopathological examination is the definitive diagnostic modality. Complete surgical excision is both diagnostic and curative, and awareness of this rare presentation is essential to prevent misdiagnosis and ensure appropriate management.