Internal jugular vein thrombosis (IJVT) is a rare but potentially lifethreatening condition which necessitates early clinical recognition and rapid administration of appropriate therapy. IJVT was associated with deep neck infection during the pre-antibiotic era. Today, trauma to the internal jugular vein from catheterization and repeated intravenous injections by drug users and direct extension of tumor which is rare are the leading causes of thrombosis. Contrast-enhanced computed tomography and magnetic resonance imaging provides an accurate diagnosis and prompts immediate therapy. Management of IJVT involves antibiotics, anticoagulation and surgical intervention.