A case of the highly placed jugular bulb in a 17-year-old girl who had complained of hearing impairment and otalgia on the right side and dizzy sensation was reported. Exploratory tympanotomy showed a blue-purple and non-pulsatile mass near the lenticular process of incus in the hypotympanum. Also, jugular venography done postoperatively showed a definite protruding of jugular bulb into the middle ear cavity. This case was reported in detail and the literature review was made.