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Adrenal insufficiency (AI) is a disorder characterised by inadequate cortisol production due to dysfunction of the adrenal glands or impairment of the hypothalamic–pituitary–adrenal (HPA) axis. It is classified as primary AI, caused by intrinsic adrenal disease, or secondary/tertiary AI, resulting from reduced adrenocorticotropic hormone (ACTH) or corticotropin harmone (CRH) secretion. Because symptoms are often non-specific, diagnosis may be delayed, increasing the risk of adrenal crisis, a potentially life-threatening complication requiring prompt recognition and treatment. 1 AI is a rare but potentially life-threatening endocrine disorder in children. Early identification and appropriate management, including prevention and prompt treatment of adrenal crisis, are essential to reduce morbidity and mortality. This updated NICE (National Institute for Health and Care Excellence) guideline, published on 28 August 2024, provides comprehensive and practical recommendations across the patient journey from diagnosis to long-term care.