Necrotizing sarcoid granulomatosis (NSG) is a rare disease characterized by granulomatous and necrotic features as well as vasculitis, and it primarily affects the lungs, with occasional extrapulmonary manifestations. The first documented case was in Jakarta, Indonesia. A 71-year-old male presented with prolonged fever, a neck mass, and multiple mediastinal lymphadenopathy. The disease was initially suspected as lung tuberculosis, but a surgical biopsy of the left thyroid lobe confirmed the NSG pattern. Treatment with oral prednisolone led to positive outcomes, as evidenced by radiological improvement at the 3-month follow-up. This case report aims to emphasize the challenges and the importance of clinician awareness in diagnosing NSG. Copyright © 2025 Gurmeet Singh et al. Case Reports in Medicine published by John Wiley & Sons Ltd.