Identification and investigation of hypoglycaemia in childhood remains an important clinical emergency. Rapid recognition and appropriate management of this clinical state continues to be important in order to prevent neurological damage or even death. The purpose of this review is to provide an update on the advances made in this area since the review by Bonham in this journal in 1993. Advances in molecular science and diagnostic techniques have assisted in understanding the mechanisms involved in the homeostasis of glucose metabolism at rest and when stressed. New disorders causing hypoglycaemia are described using the classification based upon aetiologies, which was used in Bonham's original paper. The development and use of guidelines and pre-assembled packs for investigating hypoglycaemia is also discussed.