Ectopic livers are infrequently reported in the literature. The reported size for ectopic livers range from a few millimeters up to several centimeters. They are often clinically silent and incidentally discovered during imaging of the hepatobiliary tract, regional surgical procedures or autopsy. They are predestined for benign liver diseases otherwise observed in normal livers like fatty change or develop malignancies such as hepatocellular carcinoma, in a manner analogous to the parent orthotopic liver. The presence of abnormal alpha 1-antitrypsin retention in an ectopic liver has, to our knowledge, not been reported in the literature. Hereby, we present the first reported case featuring alpha 1-antitrypsin retention in an ectopic liver attached to the fundus of the gallbladder and present the clinical, radiological and pathological findings in a caucasian woman undergoing cholecystectomy for acute cholecystitis. Special liver stains showed an alpha 1-antitrypsin retention which was confirmed immunohistochemically. Although ectopic livers are rare and usually an incidental finding, the radiologist and the surgeon should take this into the differential diagnosis of a mass attached to the gall bladder. A secondary disease should be considered by the pathologist in such a specimen and alpha 1-antitrypsin retention should be ruled out by special liver stains. Finally, such a finding should prompt the managing clinician to exclude systemic alpha-1 antitrypsin deficiency in the patient through further appropriate tests.