With the introduction of hormonal substitution therapy in the 1950s, adrenal insufficiency (AI) has been turned into a manageable disease in pregnant women. In fact, in the light of glucocorticoid replacement therapy and improved obstetric care, it is realistic to expect good maternal and fetal outcomes in patients with AI. However, there are still a number of challenges such as establishing the diagnosis of AI in pregnant women and optimizing the treatment of AI and related comorbidities prior to as well as during pregnancy. Clinical and biochemical diagnoses of a new-onset AI may be challenging because of overlapping symptoms of normal pregnancy as well as pregnancy-induced changes in cortisol values. Physiological changes occurring during pregnancy should be taken into account while adjusting the substitution therapy. The high proportion of reported adrenal crisis in pregnant women with AI highlights persistent problems in this particular clinical situation. Due to the rarity of the disease, there is no prospective data-guiding management of pregnancy in patients with known AI. The aim of this review is to summarize the maternal and fetal outcomes based on recently published case reports in patients with AI and to suggest a practical approach to diagnose and manage AI in pregnancy.