Endocrine diseases, such as diabetes mellitus and thyroid dysfunction, have been rarely associated with increased serum transaminase activity. The association of Addison's disease with abnormal liver function tests has received less attention. Addison's disease as a part of autoimmune polyglandular syndrome-1 may be associated with autoimmune hepatitis. Addison's disease may also coexist with celiac disease, an autoimmune disorder commonly associated with hypertransaminasemia. On the other hand, a number of case reports have suggested hypertransaminasemia to be one of the few diagnostic clues to the underlying adrenal insufficiency, allowing the introduction of steroid replacement and obviation of a potential adrenal crisis. We performed a thorough literature review on the prevalence and pathogenesis of hypertransaminasemia as a feature of Addison's disease in an attempt to highlight an as yet under-recognized association.