Soft tissue tumors account for less than 0.1% of colon and rectum malignant neoplasms. Epithelioid sarcoma is a subtype of undifferentiated soft tissue neoplasia that makes up 1% of this category; it is characterized by a slow progression, commonly affecting young male adults, with little or no response to chemotherapy and radiotherapy, leaving surgical resection their main therapeutic option to reduce recurrence. We here present the case of a 71-year-old male patient admitted for major rectorrhagia after evacuations and anal pain. During physical examination, a posterior anal sphincter induration was noted, which extended to the puborectal muscle. Anoscopy was performed where a thrombosed hemorrhoidal bundle was detected adjacent to a mass. In the later biopsy with immunohistochemistry, a proximal ulcer type, histological grade three epithelioid sarcoma was diagnosed. Subsequently, traditional abdominal perineal resection with colostomy was performed, resulting in favorable postoperative evolution and discharge after six days of the procedure. Keywords: soft tissue tumors; epithelioid sarcoma.
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