Background Adrenal insufficiency, the clinical manifestation of deficient, production or action of glucocorticoids with or without deficiency in mineralocorticoids and androgen. It is a life-threatening disorder. Design and Setting A retrospective, hospital-based study was conducted at King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia during the period January 1989 and December 2014. Material and Methods The medical records of children with the diagnosis of adrenal insufficiency were retrospectively reviewed. Data included age, sex, clinical presentation, and results of relevant laboratory and radiological investigations. Results During the period under review, (January 1989 and December 2014), a total of 148 patients with adrenal insufficiency were seen. Primary adrenal insufficiency was the commonest occurrence found in 84.5% of patients with the predominance of congenital adrenal hyperplasia (CAH). A diversity of other causes were also seen. Conclusion A wide spectrum of aetiological causes were noted. Congenital adrenal hyperplasia was the commonest. In Saudi Arabia, where consanguineous mating is high, these findings were not unusual.