Chordoma is defined as clinically malignant tumor originating from vestigial or residual remnants of the embryonic notochord. It is known as a slowly growing and locally invasive tumor that tends to recur after surgical removal. This tumor is very rare and, based on the anatomical predilection, there are three major groups such as cranial or spheno-occipital, vertebral and sacrococcygeal. The spheno-occipital type may extend ventrally in the direction of the nasopharynx, so it is sometimes called as nasopharyngeal chordoma. Authors experienced a case of 34 years old woman who had suffered from left side otalgia, hearing impairment, tinnitus and headache for 2 years prior to admission. She also complained of voice disturbance. The physical examination revealed smooth surfaced, firm, egg-sized tumor mass which was presented in the left side of nasopharynx with extension to the tonsillar fossa. Left ear drum showed marked bulging due to underlying mass. Paralysis of the soft palate, tongue and vocal cord on left side were also noted. Nasopharynx was explored by the transpalatal approach and dissection was done after tonsillectomy. The tumor was thick-walled cystic mass containing jelly-like amorphous substance. It was extended to the basiocciput and through the Eustachian tube into the temporal bone and middle ear cavity. The histopathological study confirmed this tumor as chordoma showing typical numerous physaliphorous cells and chondroid tissue.