Uterine schwannoma is a very rare in gynecologic oncology. We report a case of benign schwannoma in a 39-year-old woman. Case Our patient (gravida 2 para1) presented with mass on uterus. She cognized that mass 9 years ago. At that time, on transvaginal sonography, the solid and homogeneous mass's size was 3.0*4.0cm, but the size increased to 6.0*4.0 cm during 9 years. She complained any other symptoms. By transvaginal sonography, physician supposed the mass to subserosal myoma of uterus and determined laparotomy. Under general anesthesia, myomectomy was done. No ascites were detected. Both ovaries were normal. Myoma was consisted of several fragments of a gelationous and myxoid encapsulated mass, measuring 6.0 x 8.0 x 3.0 cm in aggregate and weighing 74.3g. Upon cross section, the cut surface reveals a heterogenous surface admixed with areas of hemorrhage, areas of dense yellow and areas of less dense tan color. Immunohistochemical stains and special stain were done. S100 protein, alcian blue stain reacted positive. That mass was diagnosed schwannoma. C-kit and CD34 reacted negative, so we could rule out the possibility of extragastrointesinal stromal tumor. 3 month later from operation, by follow up sonography, there was any lesions on her uterus. Discussion Schwannoma is a benign peripheral nerve sheath tumor. It is homogeneous tumors, consisting only of Schwann cells. They present in most often in the head and neck region. Uncommonly, large tumors are found in the posterior mediastinum or the retroperitoneum. Typical schwannomas can also involve visceral sites primarily, however, in the uterus is extremely rare. In this case, lesion should be considered in the differential diagnosis such as neurofibroma, leiomyoma, angiomyofibroblastoma, and desmoplastic melanoma. The absence of staining for CD34 and NFP, but they are S-100 positive, which is a marker for cells of neural crest cell origin. It supports the diagnosis of schwannoma as opposed to neurofibroma.