The authors report their experience with a case of a voluminous malignant pleomorphic histiocytoma of the anterior abdominal wall occurring in an 81-year-old female patient. She presented with a one-year history of a non-painful, poorly mobile tumefaction which had grown in size over the 2 months prior to admission. A preoperative CT scan confirmed the presence of a 10-cm mass located in the anterior abdominal wall. The patient underwent resection of the tumour and was discharged on postoperative day 15. A 12-month follow-up has so far revealed no evidence of recurrence. The authors go on to examine the diagnostic and therapeutic treatments of these rare neoplasms with reference to the international scientific literature. They confirm the validity of radiotherapy but stress the elective role of surgical treatment.