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Xanthogranuloma is a granulomatous lesion associated with histiocytic proliferation and lipid accumulation. It occurs as solitary or multiple smooth-surfaced papules or nodules. It generally appears on the skin in infancy and childhood, in which case it is known as juvenile xanthogranuloma, although a few adult-onset cases have also been reported. Adult-onset cases are known as adult-onset xanthogranuloma, and adult-onset xanthogranuloma of the tongue is extremely rare. The disease is difficult to diagnose clinically; it is instead diagnosed histopathologically in most cases. We herein report a case of adult-onset xanthogranuloma of the tongue.