Air embolism is a rare but dreaded complication of medical or surgical procedures with significant mortality. An abnormal air-to-blood pressure gradient and communication between the vein and atmosphere can lead to the venous air embolism. Here, we presented a rare case of venous air embolism just after the removal of the central venous catheters. A 59-year-old female was admitted to Peking University International Hospital suffering from neuromyelitis optica spectrum disorder. After 5 rounds of immunosorbent therapy, her hemodialysis catheter was removed. Five hours later, she suddenly experienced shortness of breath, dizziness, weakness of the lower extremities, transient loss of consciousness and seizures. Ultrasound showed a small amount of gas in the right internal jugular vein and brain CT showed scattered air accumulation in part of venous blood ducts and bilateral cavernous sinuses, which was diagnosed as air embolism. High-flow oxygen inhalation, compression of the right neck extubation site, head down and feet up left decubitus position, intravenous fluid therapy were used. The patient gradually improved and returned to normal activities after one week. To sum up, air embolism is a rare, but serious and life-threatening complication after central catheters removal. Clinicians need to be alerted and take appropriate preventive measures.