Abstract CREST syndrome, a subtype of scleroderma and Primary Biliary Cholangitis, is an autoimmune disease where the immune system is causing a progressive slow damage to the bile ducts. We report the case of a 67-year-old female patient presenting symptoms indicative of CREST syndrome. During her hospitalization in the internal medicine clinic and after the appropriate imaging and laboratory investigation the diagnosis of CREST syndrome was confirmed, and she was also diagnosed with PBC. This case adds up to a list of literature sources and several case reports which indicate the association of PBC and CREST syndrome, including the accompanied clinical symptoms and musculoskeletal manifestations of the latter.