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Introduction Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease that may progress to cirrhosis and its complications. Portal hypertension (PHT) is traditionally considered a late-stage manifestation associated with advanced fibrosis; however, evidence suggests that PHT may develop earlier and remain under-recognized. Early identification of PHT in PBC is crucial due to its prognostic and therapeutic implications. This study aimed to describe the prevalence, clinical features, management, and treatment response of PBC patients with PHT in a Moroccan cohort.Methods We conducted a retrospective study in a tertiary hepatology centre, including 64 patients with PBC and clinically significant PHT followed between 2010 and 2025. Demographic, clinical, biochemical, imaging, endoscopic, immunological, and histological data were collected. Fibrosis was assessed using transient elastography or liver biopsy. All patients received ursodeoxycholic acid (13–15 mg/kg/day), and PHT was managed according to standard clinical practice. Biochemical response was evaluated using Paris II criteria.Results Among 142 patients with PBC, 64 cases (45%) presented with clinically significant PHT. The mean age was 50.9 years, with a female predominance (sex ratio 1:3). Common symptoms included jaundice (53%), asthenia (45%), and pruritus (36%). Clinical examination revealed ascites in 48%, splenomegaly in 28%, and collateral venous circulation in 19%. Ultrasound showed features of chronic liver disease in 71%, splenomegaly in 41.9%, and ascites in 51%. Laboratory abnormalities included cytolysis (78%), cholestasis (70%), hepatocellular insufficiency (44%), and thrombocytopenia (32.8%). Endoscopy revealed esophageal varices in 64% and portal hypertensive gastropathy in 43.8%. Histological staging according to the Scheuer classification was available in 38 patients, with advanced stages (3-4) present in about 30% of cases. Fibrosis assessment in 48 patients showed advanced fibrosis (≥F3) in 33 patients. Associated autoimmune diseases were frequent, most commonly autoimmune hepatitis and autoimmune thyroiditis (23% each). Paris II criteria were available for 46 patients, of whom 47.8% achieved a good biochemical response.Conclusion Portal hypertension is a frequent complication of primary biliary cholangitis and may occur independently of advanced fibrosis. Systematic screening for PHT in PBC patients is essential for early detection and optimal management.