Schwannoma (also known as neurilemmoma) is a type of benign tumor that originates from the myelin sheath around of peripheral nerves. It is composed of schwann cells, which are specialized cells that help to insulate nerve fibers. Schwannomas are relatively rare, accounting for only 5% of all benign tumors in the perineal region. They are usually asymptomatic and are only detected when they come into contact with other organs or structures, causing symptoms such as compression. Preoperative diagnosis of schwannoma is challenging, as it is often difficult to distinguish it from other types of tumors in the same area. The final diagnosis can only be confirmed by intraoperative histology.The method of choice for benign neurilemmomas is complete removal, as they continue growth after resection. Removing part of the tumor may be an option in cases where removing the neoplasm en bloc could damage adjacent structures, such as anal sphincter, levators, rectum, vagina and urethra.Clinical case: twenty-five years old female, had complaints of a tumor in the left buttock area and discomfort in a sitting position for 3 years. Pelvic MRI with intravenous contrast revealed a tumor 6.0 × 5.1 × 9.0 cm in the left ischial region. Surgical removal of the tumor was performed.