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BACKGROUND Natural killer (NK)/T cell lymphoma is a rare and highly aggressive malignant tumor, and is a special form of non-Hodgkin's lymphoma. Although extranodal involvement is frequently found in tissues such as the skin, testicular and gastrointestinal tract etc, its presence in skeletal muscle has scarcely been reported in the literature. CASE SUMMARY We report a case of extranodal NK/T cell lymphoma with muscle swelling as the first clinical manifestation. A 42-year-old man, who initially presented with localized swelling in the dou