[Leading symptom: esophageal varices hemorrhage in a young man]

Indicating sign: Esophageal hemorrhages in adolescence. Congenital hepatic fibrosis and infantile polycystic disease of the kidneys. A 17-year-old patient with hypersplenism and signs of portal hypertension came to clinical treatment after an esophageal hemorrhage. Rare syndrome of congenital hepatic fibrosis with portal hypertension and splenomegaly, with infantile polycystic disease of the kidneys and aneurysms of the splenic artery was recognizable by means of ultrasound, laparoscopy and angiography. Therapeutically splenorenal shunt and splenectomy were carried out.