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The most common skin manifestation of type 1 neurofibromatosis is plexiform neurofibromas. Malignant peripheral nerve sheath tumors are rare and aggressive malignancies that predominantly arise from peripheral nerves and various nerve sheath cells. This report presents the case of a male patient in his late 50s who was diagnosed with malignant transformation of type 1 neurofibromatosis and treated surgically. 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