Eccrine spiradenoma is a benign tumour originating from cutaneous sweat glands. It rarely develops into a malignant tumour. A long-standing cutaneous nodule begins to enlarge rapidly. The growth is often associated with an ulceration and a change in colour. The definitive diagnosis is made after a biopsy with examination of the entire specimen in search for foci of malignant development. The appropriate therapy consists in a wide local excision, with resection of the clinically suspicious lymph nodes. Irradiation of the resection site can be useful to prevent local recurrences. The role of chemotherapy is not yet clearly defined.