Sarcoid-like granulomatous inflammation (SLGI) is defined as the development of non-necrotizing epithelioid granulomas in patients who do not meet the criteria for systemic sarcoidosis. Its occurrence is known to be linked to diverse conditions, including malignancies, infections, the use of certain drugs and inorganic substances. To the best of our knowledge, the available literature to date lacks any description regarding SLGI in a paraganglioma. The present study describes the first case of SLGI in a carotid body paraganglioma (CBP). A 54-year-old female patient presented with anterior neck swelling for 27 years without any other symptoms. An ultrasonography revealed a multinodular goiter with retrosternal extension and a solid lesion on the right side of the neck measuring 40x30x22 mm, which was suggestive of a CBP. The patient underwent a total thyroidectomy, and the right-side cervical mass was resected via another longitudinal incision. A histopathological examination of the thyroid specimen revealed findings of a multinodular goiter. Sections from the right-side cervical mass confirmed the diagnosis of CBP accompanied by multiple, well-formed, small-medium sized, non-necrotizing epithelioid granulomas associated with multinucleated giant cells, indicative of SLGI. Non-necrotizing epithelioid cell granulomas (as with SLGIs), identical to those observed in sarcoidosis, may rarely be observed in patients who do not meet the criteria of systemic sarcoidosis; however, they have been observed in association with various neoplasms. It is thus crucial to distinguish them from actual sarcoidosis, as misdiagnoses may lead to severe consequences. The presence of SLGIs accompanying a paraganglioma is an extremely rare phenomenon. Due to this, it is difficult to conclude if it confers a better prognosis or not.