Background <p>Caroli’s disease is an inherited disorder characterized by dilatation of intrahepatic bile ducts and congenital hepatic fibrosis. Caroli’s disease is complicated by choledocholithiasis, cholangitis, and portal hypertension.</p> Case presentation <p>A 28-year-old Palestinian female with 7-year history of Caroli’s disease complicated by liver cirrhosis, portal hypertension, and esophageal varices presented with hypersplenism and thrombocytopenia. She was managed with splenic artery embolization, which improved her platelet count. The splenic artery embolization was complicated by postembolization syndrome, and she was treated with antibiotics and supportive care. Two months later, she died owing to liver encephalopathy and spontaneous bacterial peritonitis.</p> Conclusion <p>This case highlights the role of splenic artery embolization in managing hypersplenism associated with Caroli’s disease. Despite improvement in platelet count, post-embolization syndrome remains a significant risk. Early liver transplantation should be considered in patients with advanced Caroli’s syndrome to improve long-term outcomes.</p>