Central venous catheterization can have many complications. A pneumothorax, hemothorax, hemomediastinum, air embolism, cardiac tamponade, and major vascular laceration are complications that can be induced by internal jugular venous catheterization. A patient with Marfan syndrome had a soft connective tissue and variable characteristic major vascular anomaly. We report a case of an acute lethal extrapleural hematoma in a 14-year old female with Marfan syndrome resulting from a laceration of the subclavian artery during internal jugular venous catheterization.