Gaucher’s disease and alpha-1 antitrypsin deficiency are genetic diseases that can cause different kinds of liver damage, but are rarely associated with cirrhosis. Here, we describe the case of a patient with both diseases who presented with cirrhosis, followed by liver failure and death. Although the interaction between these two diseases remains unclear, we suspect the involvement of an epigenetic factor in the evolution of the
aggressive liver disease. Key words: Gaucher’s disease − alpha-1 antitrypsin deficiency − liver fibrosis − cirrhosis. Abbreviations: AATD: alpha-1 antitrypsin deficiency; GCase: glucocerebrosidase enzyme; GD: Gaucher’s disease; GC: glicosilceramide.