Malignant fibrous histiocytoma (MFH) is a tumor most frequently occurring in lower and upper extremities and in retroperitoneum. This paper presents the extremely rare case of a 55-year-old male patient with a MFH of storiform pleomorphic subtype originating from the mesentery. Sonography, computed tomography as well as endoscopy and ERCP did not reveal the diagnosis. Finally the tumor was diagnosed by laparoscopy with biopsy and histological examination. Two months after diagnosis of the tumor the patient died of bronchopneumony and heart failure contracted during a generalisation of MFH.