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Extrarenal rhabdoid tumour of the vulva: diagnostic and management challenges. Extrarenal rhabdoid tumour (ERT) of vulva is a rare gynaecological neoplasm with an aggressive course and no clear management guidelines. We present the case of a 25-year-old woman with a rapidly increasing mass in right vulva suggestive of sarcoma. Wide local excision was done. Histopathology examination revealed ERT of vulva. Six weeks later she manifested lung metastases. Despite adjuvant chemotherapy, the disease progressed and she died 8 months later. We review the literature and briefly discuss the epidemiology, treatment approaches, prognostic factors and expected outcomes of this rare disease.