High jugular bulb is a relatively common anatomical variation that usually has a dormant clinical course. However, if a high jugular bulb is undiagnosed prior to an otological procedure or a skull base surgery, the operation may become unfeasible due to exposure difficulty and profuse bleeding. Rates of complication and morbidity will drastically increase in such a circumstance. In a patient with total sensorineural hearing loss due to vestibular schwannoma, a co-existing high jugular bulb was detected. Complete resection was performed via translabyrinthine approach. High jugular bulbus must be carefully investigated prior to skull base surgery and surgical planning must be done accordingly.