Primary adrenal insufficiency is relatively common in children, but primary presentation with acute shock/adrenal crisis is rare. Congenital adrenal hyperplasia (CAH) is the most common cause in children and is part of the newborn diagnostic screening programme in Norway.We admitted a schoolgirl with a history of nausea and morning sickness for the previous three weeks. Shortly after admission she was somnolent, tachycardic (148 beats per minute) and pale. After initial administration of a 500 ml bolus of Ringers acetate she was transmitted to the ICU. She responded poorly on IV fluids and epinephrine alone. Arterial blood gas test showed metabolic acidosis with hyponatraemia 122 mmol/L, hyperkalaemia 5,6 mmol/L and hypoglycaemia 2,2 mmol/L, and adrenal insufficiency was suspected. She responded well to treatment with hydrocortisone and was transferred to a university hospital for further examination and diagnosis.Acute adrenal crisis is a rare primary presentation of adrenal insufficiency, especially in the paediatric population. It is an important differential diagnosis of shock and has high morbidity and mortality.