Recently, two cases of Banteay’s disease, a rare liver disease, were diagnosed in the Third Department of Hepatobiliary Medicine using modern advanced technology and with the assistance of sister departments and hospitals. Two patients are waiting for surgical treatment. Banti’s disease is mainly characterized by portal hypertension, marked splenomegaly with hypersplenism and anemia, while liver function is basically normal. The pathological changes are mainly portal vein fibrosis, destruction of the terminal branches of the intrahepatic portal vein, and atrophy of the liver parenchyma. The hemodynamic changes are pre-sinusoidal portal hypertension in the liver, i.e., a significant increase in portal venous pressure with normal wedge pressure in the hepatic veins and increased blood flow in the splenic and portal veins. The disease is clinically rare, its etiology is unknown, and it has been rarely reported in China. Two patients, one a 30-year-old male, presented with splenomegaly and hypersplenism, but no significant abnormalities in liver function, and laboratory tests excluded the possibility of various hepatitis. The other was a 57-year-old female with a similar clinical presentation and extreme anemia, which also ruled out various hepatitis possibilities. Both patients visited several hospitals, but neither was able to identify the cause. After consulting with the Department of Hepatobiliary Medicine, Chief Li Hucheng led attending physicians Huang Hui, Wang Rui Guan, and Wu Tian Tian after detailed medical history and systematic physical examination, and after repeated discussions and review of data, concluded that the patient might be suffering from a rare liver disease. After DSA and 320-row CT excluded the portal vein trunk lesion, Director of Ultrasound Department Cao Bingsheng performed liver angiography under ultrasound and liver biopsy. The biopsy tissue was diagnosed as Banteay’s disease after consultation with Director Li Ning of our hospital, Directors Zhao Xiancheng and Zhou Guangde of 302 Hospital and Senior Pathologist Wang Tai-ling of China-Japan Friendship Hospital, who is over 80 years old. According to Director Zhao, the disease is relatively rare even in such a specialized hospital for liver disease as 302, and also requires multi-disciplinary collaboration between clinical and imaging, case and other disciplines and repeated consultations to determine. Diagnostic points of Banteay’s disease: 1. Unexplained splenomegaly, anemia, portal hypertension, except cirrhosis, blood diseases, parasitic diseases of the hepatobiliary system, hepatic and portal vein obstruction and congenital liver fibrosis, etc. In other words, the clinical diagnosis of Banteay’s disease can be established by excluding the above related diseases. 2.Reduction of more than one blood component. 3.Liver function test is normal or near normal. 4, Ultrasound, CT or isotope examination of the spleen has dilated portal vein and splenic vein, increased blood flow, and splenomegaly. Smooth liver surface with uniform texture and no atrophy is not suggestive of cirrhosis. 5.Endoscopy or X-ray confirms the presence of upper gastrointestinal varices. 6.Hepatic vein cannulation shows open hepatic veins, WHVP is normal or mildly elevated, and direct portal venous pressure measurement is greater than 20 mmHg. 7.Laparoscopy suggests no manifestation of cirrhosis on the liver surface; liver biopsy shows portal fibrosis, but no cirrhosis. For the treatment of this disease, early splenectomy can achieve good results, while late stage will show atrophy of the liver and require liver transplantation. In the past year, the Department of Hepatobiliary Surgery has been practicing hard, focusing on consolidating the foundation, and vigorously strengthening the software, and has continuously diagnosed Gaucher’s disease, Banteay’s disease, immune liver disease, and liver iron deposition, which have never been clearly diagnosed or are very difficult to be clearly diagnosed in our hospital in the past, so that our hospital has advanced a great step in the diagnosis and treatment of rare liver diseases.