Pulsatile tinnitus of venous origin is a relatively rare diagnosis. When it originates from turbulent flow within the transverse-sigmoid-jugular system, the surgeons are often tend to block tinnitus creating venous flow. In the presence of a contralateral underdeveloped venous system surgery, internal jugular vein ligation may lead to fatal consequences. We report a 30-year-old female patient with venous pulsatile tinnitus and contralateral hypoplastic venous system of the brain. In the planning of any surgical method having a major interference with the venous blood flow of the brain, the surgeon should make necessary measures to insure an adequate contralateral venous system.