Cavernous transformation of the portal vein occurs with long-standing portal vein thrombosis (PVT) because of the development and dilatation of multiple small vessels in and around the recanalising main portal vein. Thrombosis and occlusion of the portal vein leads to portal hypertension with enlarged spleen and the development of porto-systemic collaterals. The main clinical presentation is gastroesophageal variceal bleeding and hematologic abnormalities due to splenomegaly (hyperspleenismus-pancytopenia). We described the young patient with splenomegaly and extensive cavernous transformation of portal vein. The patient had thrombosis portal vein in early childhood and massive bleeding from large oesophageal varices at age 13. Full clinical evaluation is required because of abdominal pain. The liver is histologicaly and functionally normal. Diagnosis of cavernous transformation of the portal vein is confirmed by abdominal ultrasography, color Doppler ultrasonography and CT angiography. Oesophagogastroscopy reveals almost complete reduction of oesophageal varices, but confirms portal gastropathy as a source of patient's complaints. Natural course of PVT in this patient shows possibility of full reduction of oesophageal varices, but still the presence of different consequences of portal hypertension.