As early as 1970, Murray-Lyon et al. described the association between primary biliary cholangitis (PBC) and CREST (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome (Murray-Lyon et al. 1970). One year later, Reynolds et al. represented six women with several presentations in favor of PBC as well as CREST syndrome and defined Reynolds syndrome for the first time (Reynolds et al. 1971). As a multisystemic disease, Reynolds’ syndrome is identified by the coexistence of progressive systemic sclerosis or its variants and PBC (Cabane 2010; Jakez-Ocampo et al. 2007; Kiyani and Ursu 2017). Systemic sclerosis refers to a connective tissue disorder defined by vasculopathy, fibrosis, and the presence of autoantibodies. PBC, a cholestatic liver disease with autoimmune manifestations, is indicated by destroyed intrahepatic bile ducts contributing to liver cirrhosis if the treatment fails (David et al. 2021). The presence of PBC is seen in approximately 2.5–3% of those suffering from systemic sclerosis. Moreover, scleroderma is reported to arise in approximately 3–17% of patients with PBC (Gharbi et al. 2019). There is a 5–10% association between primary biliary cholangitis and systemic sclerosis (Aldás and Torres 2018). Reynolds syndrome is more prevalent among women and most cases primarily manifest with CREST syndrome (Aldás and Torres 2018). The Reynolds syndrome’s picture can range from mild asymptomatic transaminase abnormalities to cirrhosis with severe hepatic insufficiency (Aldás and Torres 2018). According to previous reports, this syndrome is mainly diagnosed in adulthood (David et al. 2021; Gaudy-Marqueste et al. 2010; Gharbi et al. 2019; Harisankar et al. 2020; Jakez-Ocampo et al. 2007; Reynolds et al. 1971).