Malignant eccrine spiradenoma is an extremely rare skin tumor of sweat gland origin. The available literature data indicates that spiradenocarcinoma nearly uniformly arises from a benign preexisting spiradenoma. Etiology is unknown but previous trauma is believed being an implicated factor. The article presents the clinical observation of a 34-year-old female patient, diagnosed with cutaneous spiradenocarcinoma of the left thigh, treated multidisciplinary: surgery and chemoradiotherapy. The presentation includes clinical, histopathological and therapeutic diagnosis aspects, arisen difficulties of histological diagnosis and literature data regarding treatment options for this type of tumor. In this case, surgery followed by second surgery intervention was performed due to the continuing development of local disease. After surgery, the patient performed external beam radiotherapy (EBRT) to tumoral bed (total dose of 50 Gy) and inguinal lymph nodes (total dose of 50 Gy), followed by six cycles of chemotherapy (CMT) (Carboplatin + Paclitaxel) with complete response. At the moment, the patient shows no evidence of recurrence or metastatic disease during the follow-up.