Background: Autoimmune cholestatic liver diseases (ACLDs) are recognized risk factors for liver cirrhosis and end stage liver disease. Data on ACLD from Saudi Arabia and other Middle Eastern countries are limited to case reports. The aim of this study was to evaluate the patterns, laboratory findings and long term outcomes of ACLD patients treated at a tertiary academic medical center in Saudi Arabia. Methods: This was a retrospective study of all patients diagnosed with ACLD from January 2016 to January 2020. For all patients, demographic data, laboratory data, and the Fibrosis score assessed by Transient elastography using the Fibroscan device were obtained. We also evaluated treatment response and long term outcomes for all patients. Results: Eighteen patients were diagnosed with ACLD during the study period. This included 9 patients with primary biliary cirrhosis (PBC), 3 patients with primary sclerosing cholangitis (PSC), and 3 patients with autoimmune hepatitis (AIH)-PBC overlap. Antimitochondrial antibody was positive in all PBC patients. One third of patients had cirrhosis. Treatment with ursodeoxycholic acid (UDC) was able to achieve disease control in about 90% of PBC patients for approximately 10 years. Only one patient without PBC had complete response to treatment with UDC and immunosuppression medication. Two patients (11%) with advanced PSC died. In conclusion: ACLDs are an important cause of chronic liver disease. Early recognition of PBC and initiation of treatment can delay disease progression in the majority of PBC patients. Achieving disease control with treatment is more difficult in patients with PSC and overlap syndromes.