The earliest references to association between mechanical jaundice and non-cirrhotic portal hypertension (NCPH) are by Fraser et al. (1944) and Gibson et al. (1965). The term “portal hypertension biliopathy” was proposed by Dhiman et al. (1999). The incidence of asymptomatic form of portal hypertension biliopathy (PHB) in patients with NCPH ranges from 81% to 100% that is significantly higher compared to patients with hepatic block (0—33%). Symptomatic form of PHB occurs only in 5—33% of patients. Any intervention on biliary tract in patients with PHB may result in significant blood loss due to coagulopathy. Currently, there is no consensus on optimal treatment algorithm for patients with symptomatic PHB. The authors present several patients with PHB complicated by mechanical jaundice and variants for correction of this complication.