A 7-year-old boy was found to have hearing loss on the left side on school screening. On otolaryngology examination he was noted to have a vascular mass behind the tympanic membrane, located inferiorly. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) revealed a dehiscent high jugular bulb. He underwent surgical decompression of the jugular bulb. Two weeks after surgery, he complained of headache and diplopia and was noted to have papilledema and a sixth nerve palsy without visual loss. Cranial MRI scan revealed thrombosis of the left internal jugular vein, transverse and sigmoid sinus. There was no cerebral venous infarct. He was treated with oral acetazolamide and anticoagulation. Two months later he was symptomatically better, neurologically intact with resolved sixth nerve palsy and markedly improved optic disc edema. To our knowledge this is the first reported case of venous thrombosis following jugular bulb surgery in the English language ophthalmologic literature.