Radioembolization with yttrium-90 (Y-90) microspheres is primarily used in liver tumor patients with suboptimal response to chemotherapy. Radioembolization-induced liver disease (REILD) is a potentially fatal complication of Y-90 radioembolization (Y-90 RE). The clinical presentation of REILD mimics sinusoidal obstructive syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), sharing features including hyperbilirubinemia, coagulopathy, and ascites. While SOS/VOD in pediatric bone marrow transplant recipients is commonly treated with defibrotide, no standardized guidelines exist for the treatment of REILD. We present a case of a pediatric liver transplant recipient who developed REILD after Y-90 RE and was successfully treated with defibrotide.