Abstract
A 27-year-old woman was confirmed to have extraabdominal desmoid-type fibromatosis. Desmoid-type fibromatosis is a very rare connective tissue neoplasm with the extraabdominal type even more rare. It is most commonly found in proximal structures such as shoulders, neck, chest, and extremities. There are few case reports for desmoid-type fibromatosis located in paraspinal soft tissue. We report a case of desmoid-type fibromatosis mimicking a myxofibrosarcoma. Teaching Point: Even if there are suggestive findings for malignant soft tissue tumor on radiologic evaluation, histological confirmation is necessary before surgical treatment.