Background: Primary adrenal failure (PAF) can be congenital or acquired. Clinical manifestations are not specific and require a high index of suspicion. Objective: Report 2 cases of PAF in children and review the literature. Case-reports: 2 children 9 and 6 years-old with asthenia, fatigue, anorexia, abdominal pain, weigh loss, vomits, salt craving, weakness and increase skin and mucosal pigmentation. Both cases had glucocorticoids and mineralocorticoids deficiency, with positive anti-adrenal antibodies that confirmed an autoimmune etiology. Conclusions: PAF is uncommon in children; however, it is a potentially fatal disease when unrecognized and untreated