A 46-year-old man was referred to us because of a nodule of 20 years' duration on his lumbar region which gradually increased in size since several months ago. Axillary and inguinal lymph nodes were swollen. Microscopically, nests of tumor cells were not continuous to the epidermis. In the upper dermis, the tumor cells without anaplasia surrounded a large area of necrosis and tended to form luminal structures and whorls of squamoid cells. In the deeper portion of the dermis, the tumor cells possessed large, obviously atypical nuclei. A diagnosis of malignant transformation of intradermal acrospiroma was made. Soon after the tumor excision and lymph node dissection, a local recurrence and a lymph node metastasis to the supraclavicular region was noticed. In spite of radiation and combination chemotherapy, the patient died of metastases to multiple organs 7 months after admission.