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Malignant acrospiroma is an uncommon tumor of the eccrine sweat gland. In contrast to its relatively more common benign counterpart, malignant acrospiroma is highly invasive, often with significant lymphatic and distant metastasis. The establishment of this diagnosis is difficult on both clinical and histopatho-logic grounds. After diagnosis, wide surgical excision is warranted to completely extirpate these lesions. We report the case of a 66-year-old female with a recurrent malignant acrospiroma. This patient's tumor was removed by wide radical resection, including chest wall excision, followed by reconstructive surgery and radiotherapy. After 16 months there is no evidence of local recurrence or distant metastasis.