A 31-year-old man presented with malignant peritoneal mesothelioma. His past medical history was uneventful, specifically there was no personal or environmental asbestos exposure. The patient was treated by complete tumor resection including peritonectomy. Because of the histologic diagnosis of a low malignant, unusual highly differentiated epithelioid tumor with very low proliferative activity a postoperative chemotherapy was not administered. After a follow-up of 20 months, the patient is in excellent clinical condition and there is no evidence of disease by computed tomography. The present paper reports a case of this rare variant of malignant peritoneal mesothelioma.