A case of lymphomatoid granulomatosis presenting as midline granuloma has been reported because of the rarity and rapidly fatal course of the disease. The lesion primarily occupied the oropharynx, affecting the posterior pharyngeal wall, uvula, and posterior third of the tongue and extending on to the larynx to invade arytenoid cartilage, true and false vocal cords, and piriform fossa on the right side, with associated intestinal involvement.
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