Portal vein cavernous degeneration is a rare disease that has gradually increased in recent years as people’s standard of living has improved and their diet and living environment have changed. There are two types of portal spongiform degeneration: primary (common in children) and secondary (common in adults). The occurrence of portal vein cavernous degeneration can cause prehepatic portal hypertension, which results in splenomegaly, ascites, and vomiting of blood, and there is no uniform approach to the management of this disease. In portal hypertension caused by portal vein cavernous degeneration, considerable caution should be exercised if splenectomy is performed, because the disease itself is prone to portal vein thrombosis, where the blood is in a hypercoagulable state, and splenectomy can aggravate portal vein thrombosis and lead to serious consequences. According to our experience, patients with portal hypertension esophageal varices should take different treatment measures according to the specific extent of portal vein thrombosis and cavernous degeneration. In general, minimally invasive methods should be used to control and prevent bleeding as much as possible, and it is not appropriate to perform various surgeries in a hurry.