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The diagnosis of cholangitis should be made in the presence of chronic cholestasis, after ruling out chronic viral hepatitis and medication use, and provided hepatic ultrasound is normal. The clinical signs of cholangitis are not specific and include asthenia and pruritus. Anti-mitochondrial antibody (AMA) testing is the first key step in diagnosis. When positive, a diagnosis of primary biliary cholangitis (PBC) is made. PBC is a destructive autoimmune disease of the small bile ducts, mainly affecting women aged around 50. First-line treatment with ursodesoxycholic acid (UDCA) slows the progression to cirrhosis and its complications. When anti-mitochondrial antibodies are negative, cholangio-MRI is recommended to identify bile duct strictures, which may suggest a diagnosis of primary sclerosing cholangitis (PSC). This inflammatory disease of the large bile ducts tends to affect men aged around 40 and is very frequently associated with an IBD. Treatment of PSC is also based on UDCA. Complications of PSC include angiocholitis, cholangiocarcinoma, and cirrhosis with a risk of decompensation. When associated with IBD, there is also an increased risk of colon cancer, justifying annual ultrasound screening of the bile ducts as well as annual colon screening by colonoscopy.