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Malignant triton tumor (MTT) is a variant of the peripheral nerve sheath tumor. It is very uncommon but shows an aggressive course and limited survival. Half of the cases present symptoms related to neurofibromatosis type 1 disease. There is no standardized treatment, but multimodal approach is the best option. We present the case of a primary MTT located in the kidney, in a 43-year-old woman who received neoadjuvant chemotherapy as first-line treatment followed by surgical resection and adjuvant chemotherapy.

No abstract