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Introduction Portal cavernoma (PC), a vascular transformation secondary to portal vein thrombosis, can cause portal cavernoma cholangiopathy (PCC): a biliary obstruction due to peribiliary collaterals. While PC is well-described, PCC remains understudied, especially in non-cirrhotic populations.Our aim was to characterize PCC in a PC cohort, focusing on clinical, imaging, and treatment aspects.Methods This retrospective single-center study included 31 PCC cases among 73 PC patients. Diagnosis was based on CP-MRI using Barcelona (biliary ductal changes) and Shandra (ductoparenchymal features) grading systems. Data were analyzed descriptively.Results Our cohort of 31 PCC patients showed female predominance (74.2%), median age 43 years (range: 20–69). Comorbidities included diabetes (16%), tuberculosis (9.6%), celiac disease, and liver conditions (steatosis, hepatitis C virus). Prior surgeries were frequent: cholecystectomy (16.1%), hysterectomy (6.5%), splenectomy (3.2%).Symptoms included upper gastrointestinal bleeding (45.2%), biliary colic (29%), anemia signs (25.8%), and jaundice (19.4%). Abdominal distension (9.7%) was also reported, often reflecting advanced disease.Physical examination showed signs of portal hypertension: splenomegaly (45.2%), collateral veins (29%).Laboratory analysis identified cholestasis (48.4%) and cytolysis (4.9%), overlapping in 29%, and pancytopenia (6.5%).Ultrasound (n=31) found signs of portal hypertension: splenomegaly (80.6%), collaterals (71%), and chronic liver disease (51.6%). Ascites (9.7%) and steatosis (6.5%) were rare. The ‘honeycomb’ venous network (9.7%) and porto-portal collaterals (45%) were distinctive. PC diagnosis was confirmed by contrast-enhanced CT-scan.CP-MRI universally detected biliary abnormalities, with disease severity classified as follows: Barcelona Grade I (isolated ductal dilation without strictures) in 9.7%, Grade II (focal strictures without parenchymal atrophy) in 6.5% and Grade III (diffuse strictures with lobar atrophy/cirrhosis) in 83.9%. Using Shandra classification, disease progression was categorized as Grade I (peripheral dilation only) in 16.1%, Grade II (central+peripheral dilation with mild irregularities) in 32.3% and Grade III (severe ductoparenchymal distortion) in 51.6%.The underlying thrombosis causes were mainly thrombophilia (60%), especially protein C/S deficiency (41.9%), followed by myeloproliferative (19.4%) and autoimmune disorders (16.1%).Liver-related events occured in 51.6%, mainly hemorragic (81.3%) vs. ascitic (18.8%). Notably, one patient had both.Treatment included anticoagulation (64.5%; rivaroxaban: 80%, VKAs: 20%) and beta-blockers (61.3%; propranolol: 52.6%, atenolol: 47.4%). Diuretics were added in 26.3% for ascites management. One patient developed grade III Tokyo cholangitis and died from septic shock after biliary stent placement.Conclusions Non-cirrhotic PCC is a common, severe and under-recognized PC complication with female predominance and frequent thrombophilia. The high rate of advanced biliary involvement (Barcelona III in 83.9%) highlights the importance of routine CP-MRI. Early diagnosis and a multidisciplinary approach are essential to manage complications and improve patient outcomes.