Internal jugular vein thrombosis (IJVT) is a vascular disorder that results from various clinical conditions. The vast majority of IJVT are considered to be complications of central venous catheterization. The list of associated conditions must also be expanded and should include the following: mediastinal tumors, low antithrombin III levels, tumors associated with hypercoagulability, previous central venous catheterization, neck surgery, intravenous drug abuse, neoplasms of the head and neck, Hughes-Stovin syndrome, which is associated with pseudotumor cerebri and seizures in children, and other conditions such as local inflammation from radiation therapy or sepsis and infective cervical adenopathy. There are several cases in the literature related to spontaneous IJVT. Spontaneous IJVT may occur when there are no apparent predisposing mechanical or inflammatory causes although a few of these patients may harbor an occult neoplasm.