We report a neonate with vein of Galen malformation (VGM) who developed congestive heart failure (CHF) early after birth. Serial changes in plasma B-type natriuretic peptide (BNP) following an endovascular embolization procedure for VGM were mirrored in his clinical CHF status. The plasma BNP level markedly increased to 1800 pg/ml (normal, <100 pg/ml) in accordance with the severity of CHF. It rapidly decreased to 356 pg/ml during the first week after endovascular embolization for VGM. In the following 3 weeks there was an unexpected upward trend in plasma BNP despite echocardiography revealing normal biventricular function. After additional evaluation and treatment for CHF, BNP decreased again and the patient’s clinical status concurrently improved. The patient was discharged with a normal BNP level. Monitoring serial plasma BNP provides valuable information regarding the need for additional evaluation or treatment of newborns with CHF and may be used to document improvement.