Background: Hypoglycaemia is common in preterm and intrauterine growth retarded (IUGR) newborns. Although preterm and IUGR infants have limited adipose tissue stores, the role of adipose tissue and the associated adipocytokines in glucose physiology is not known. Aim: We report the case of a premature intrauterine growth retarded infant who had poor weight gain for the first 6 weeks of life and then developed severe hypoinsulinaemic hypoketotic hypoglycaemia. Results: There was markedly reduced adiposity with low serum leptin and adiponectin levels. Total energy expenditure and body composition measurements showed that body fat as a percentage of weight averaged 7% at 20 weeks and 28% at 28 weeks. At 20 weeks of age, the patient was equivalent to a deficit of >2 SD scores of body fat, but average fatness by 28 weeks. The hypoglycaemia completely resolved when the patient started gaining weight with an increase in fat mass and a concomitant increase in serum leptin and adiponectin level. Conclusions: Although the precise mechanism of this patient’s severe hypoglycaemia is unclear, further studies are required to understand the role of adipose tissue and adipocytokines in glucose homeostasis in preterm and IUGR infants.