The neurilemmoma, known to be originated from the sheath of Schwann cells, is a benign and solitary tumor which may occur from any nerve-sheath of the peripheral, sympathetic and cranial nerves except optic and olfactory nerves, which have no nerve-sheath themselves. Although it occurs most frequently in the head and neck region, the neurilemmoma of the tongue is rare. Recently, we have experienced a case of neurilemmoma in the base of the tongue in a 22-year-old man. Now we report the case with brief literature review.