A 21-year-old man presented with a sausage shaped mass lesion, located in the right anterolateral region of the neck. He had been operated on for hydrocephalus and thoraco-lumbar myelomeningocele, at the age of 10 days and 2 months, respectively. Although he was asymptomatic at the age of 10 years, ventriculoatrial (VA) shunt was considered non-functional. Doppler ultrasound showed thrombosis of the right internal jugular vein. Cervical magnetic resonance imaging displayed thrombosis and thrombophilebitis of the right internal jugular vein with loss of flow pattern. VA shunt was removed under general anesthesia and shunt material sent for culture. Staphylococcus aureus grew from the culture of shunt material. A cephalosporin (cefuroxime axetil) was administered and the neck mass disappeared in 4 weeks. He has been symptom free for the last 5 years. VA shunts for hydrocephalus have some well-defined specific complications. In this paper, a case with VA shunt related thrombosis and thrombophilebitis of internal jugular vein was presented and management of VA shunt related cardiovascular complications were discussed. (C) 2004 Elsevier B.V. All rights reserved.