Malignant mixed tumor (MMT) is a rare malignant tumor originated from sweat gland cells. As MMT tends to recur after inadequate excision, an early wide excision with a broad margin thought that the most optimal treatment. A 78-year-old man presented with a skin-colored nodule on the right 2nd metacarpo-phalangeal joint with duration of three years. The nodule had been excised 3 times in other hospital before visiting us. However, lesion had recurred within the incision line. Histopathologic examination showed chondroid stroma with cords of cells forming glandular structures with increased mitotic activity and cellular atypia. Mohs microscopic surgery (MMS) was performed under the diagnosis of MMT. After two stages of MMS, a tumor-free plane was achieved with a 6x5 cm defect and tendon was preserved. The patient was transferred to plastic surgery department and the defect was reconstructed using skin graft. A 10 month follow-up revealed no local recurrence or metastasis. MMT tends to show unclear clinical margin, moreover hand is hard to secure sufficient margin. The aims of MMS are to preserve normal tissue and completely remove tumor, MMS could be a good treatment option for MMT of the hand that needs maximal tissue preservation. And the distinctive histology of MMT leads itself well to MMS to achieve complete tumor extirpation. So, we herein report a case of MMT of the skin on the hand dorsum treated with MMS.