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The purpose of this article is to discuss the clinical and imaging findings of primary sclerosing cholangitis and its cholestatic mimickers, with a focus on MR cholangiopancreatography (MRCP) utilization and other characteristic MRI imaging findings. Primary sclerosing cholangitis is a rare idiopathic disease of the liver associated with inflammatory bowel disease which results in damage to the bile ducts and liver parenchyma. Although the etiology of primary sclerosing cholangitis is not completely understood, the disorder is thought to be a result of multiple environmental factors. Genetic factors are likewise important given that multiple genes have been linked to primary sclerosing cholangitis. While primary sclerosing cholangitis shares many clinical and imaging characteristics with other cholestatic diseases, the disorder demonstrates unique and distinguishing features. Discriminating between the similarities and differences on MRI with MRCP allows accurate clinical and radiologic diagnosis, facilitating appropriate management with improved patient outcomes.