Pulmonary hypertension is known as one of most serious complication of ventriculoatrial shunts. There is a long latency period between ventriculoatrial shunt implantation and development of pulmonary hypertension. Because of this latency and relative rarity of pulmonary hypertension in the general population, clinicians may misdiagnosis these patients. A young male patient was referred to our clinic with suspect of an atrial septal defect from an outside hospital. He had a ventriculoatrial shunt because of hydrocephalus. We tought the clinical diagnosis as pulmonary hypertension due to ventriculoatrial shunt according to the his clinical history, ECG and echocardiography findings. The pulmonary perfusion scintigraphy findings supported the diagnosis. His shunt was replaced to the ventriculoperitoneal shunt by Brain Surgery Clinic.