The co-occurrence of autoimmune hepatitis (AIH) and pernicious anemia (PA) is extremely rare. We present a case of a 70-year-old woman with AIH who presented for the evaluation of poor appetite and weight loss. Laboratory studies were significant for microcytic anemia, B12, and iron deficiency. Esophagogastroduodenoscopy showed diffuse gastric mucosal atrophy, and the pathology from the body of the stomach showed chronic gastritis. Additional testing was positive for parietal cell antibody and intrinsic factor blocking antibody, confirming the diagnosis of PA. To the best of our knowledge, there is only one documented case of AIH associated with PA.