Introduction Addison’s disease is a rare chronic illness caused by adrenocortical insufficiency. The symptoms and signs of Addison’s disease are mostly non-specific, but characteristic hyperpigmentation and salt craving are common.

Patients and Methods A retrospective study over a period of 18 years (1990–2007). We verify the diagnosis of Addison’s disease if the morning serum cortisol level is low, the adrenocorticotrophic hormone (ACTH) concentration is high and/or the tetracosactide stimulation test is negative.

Results We included 22 children with Addison’s disease. They were 15 boys and seven girls. The middle age when the diagnosis was carried out was 4 years (6 days to 11 years). Melanodermy was constant in all the children at the physical examination. A psychomotor delay was noted in one case. The average morning serum cortisol level was 57.3 ng/ml. The ACTH measurement obtained in 11 cases was 2322 ng/ml (84.7–9949). The tetracosactide stimulation test carried out in six patients was negative in all cases. The aetiological investigation enabled us to retain the diagnosis of Allogrove syndrome in eight children, an autoimmune destruction of the adrenal gland in three cases, a polyendocrine syndrome in one case, adrenoleucodystrophy in one case, in the other cases the aetiology of Addison’s disease remained unknown. Average follow-up was 4 years (3 months to 13 years), 13 children were still followed. Acute adrenal insufficiency, precipitated by acute infections, was noted in four patients. It caused death in two cases.

Conclusion Addison’s disease is a rare illness. Acute adrenal insufficiency must be provided by an adequate treatment prescribed without delay.