Advanced cystic fibrosis liver disease (aCFLD) with portal hypertension develops in a minority of people (5-7.5%) with Cystic Fibrosis (CF), necessitating surgical interventions, including portosystemic shunting, gastrostomy tube placement, and in some cases, liver transplantation. This manuscript also aims to explore the different endoscopic, endovascular and surgical procedures needed to diagnose and manage aCFLD, including liver biopsy, endoscopic retrograde cholangiopancreatography, intraoperative cholangiograms, hepatic venous pressure gradient measurement and portosystemic shunt procedures in patients with aCFLD. By utilizing advanced diagnostic and surgical techniques, healthcare professionals can effectively manage aCFLD and improve patient outcomes.