Malignant eccrine spiradenoma (MES) is an extremely rare cutaneous malignant tumour. An 86-year-old man presented at our hospital with an enlarging tumour on the dorsum of the left hand. An excisional biopsy was taken and histological examination showed a solid island of cells of two distinct types: cells with abundant cytoplasm and oval vesicular nuclei, and small round cells with less cytoplasm and hyperchromatic nuclei with a high frequency of mitosis. We diagnosed this tumour as MES. Although we did not perform further treatment because of the patient's age, there was no sign of recurrence or metastasis in the 2 years of follow-up after excisional biopsy. We reviewed cases of malignant eccrine spiradenoma in the English and Japanese literature and found that 'sarcomatous' or 'squamous' change in histopathology was significantly correlated with a poorer prognosis. It is therefore important for treatment planning to evaluate the entire specimen histologically.