<p>Ossifying fibromyxoid tumor is a rare soft tissue mesenchymal neoplasm of unclear lineage and intermediate malignant potential, primarily arising in subcutaneous tissues of the limbs, head and neck, or trunk. Ossifying fibromyxoid tumors can be classified as typical, atypical, or malignant. While, most ossifying fibromyxoid tumors exhibit benign behavior, a subset demonstrates malignant tendencies, with a potential for local recurrence and distant metastasis. We report a case of an atypical ossifying fibromyxoid tumor in the soft tissue of the right elbow, characterized by multiple local recurrences and pulmonary metastases. This report details the clinical presentation, radiological findings, histopathological features, and therapeutic interventions, highlighting the challenges in managing this aggressive variant.</p>