INTRODUCTION: The patients with external ventricular drains (EVD) are at risk of developing meningitis either bacterial or aseptic. Aseptic meningitis is rarely caused by degradation of blood components in the ventricular and subarachnoid space. We report a case of aseptic meningitis in a preterm infant who underwent EVD with intraventricular hemorrhage (IVH). CASE: A male infant was born by an emergent Cesarean section with placental abruption and twin pregnancy at the gestational age 31 weeks and 3 days. The birth weight was 1,630 g, and the Apgar scores were 6 and 8 at 1 and 5 minutes, respectively. After birth, the baby was intensively treated with respiratory distress syndrome, amniotic fluid aspiration, persistent pulmonary hypertension, and IVH in the NICU. In this patient, IVH was gradually worsening over time. So ultrasound-guided EVD was performed at the bedside on the 22nd day, and the CSF was drained about 70cc of for 4 days and the catheter was removed. The patient had intermittent fever greater than 38℃ and an elevated CRP on 4th days after the removal of EVD, and a lumbar puncture was performed. CSF examination showed a pleocytosis (polymorphonuclear leukocytes: 131/μL, lymphocytes: 13/μL), and a decreased glucose level (11 mg/dL), but the pathogen did not grow in the culture. After 3 days, abnormal CSF findings and elevated CRP levels were persisted, but we discontinued an empirical antibiotic therapy at 5 days. At follow up 2 weeks later, the CSF study was normal findings except an elevated level of protein, but brain MRI showed enlarged ventricles with diffuse ependymal enhancement suggesting ventriculitis. CONCLUSION: Aseptic meningitis can be complicated in patients who underwent EVD with IVH. If the CSF is sterile, empirical antibiotics should be discontinued soon.