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Epithelioid sarcoma (ES) is a rare mesenchymal soft tissue tumor with an epithelioid pattern that often occurs in the extremities of young people and presents in a banal fashion.Its significance is underscored by the inherent propensity for local recurrence, regional lymph node involvement, and distant metastases.ES presents as nodules or skin ulcers,and is often misdiagnosed as benign granulomatous lesions.ES is also often confused with other malignancies, such as squamous cell carcinoma and synovial sarcoma.is the treatment of choice;indeed, with marginal resection, ES has a recurrence rate of up to80%.Accordingly, wide resection or tumor bed resection is required despite the dysfunction and morbidity associated with such aggressive treatment.ES spreads proximally in the same limb, distant to the original tumor, leading some to consider less radical treatments for local control.Amputation is a possibility in the event of multiple recurrences or if loss of function is minor.To date, there is no evidence that chemotherapy or radiotherapy prolongs survival or local control to a meaningful degree.Here we review our experiences on the clinical management of ES.