Granular cell tumor is a rare kind of neoplasm 1-2 % malign derived from Schwann cells any site in the body. Oral cavity, skin and subcutaneous tissue are most frequently involved while only five to eight per cent of all granular cell tumors develop in gastrointestinal system. The reported case is 66 years old male patient, with a malignant granular cell tumor in jejenum which is, to our best knowledge, first in the literature.