A low-grade fibromyxoid sarcoma is a rare soft tissue tumour that has a tendency to develop in the deep soft tissue of young adults with potential for local recurrence or distant metastasis. We describe a 40-year-old female patient with a low-grade fibromyxoid sarcoma of the shoulder that had been excised twice in the past and then had recurred after a few months. A wide resection of this tumour with flap reconstruction was performed followed by radiation to the area. The patient had no evidence of local recurrence or distant metastasis at 2 years after surgery.

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