Congenital hyperinsulinism (CHI) is a rare disorder of hypoglycaemia in children due to excessive and dysregulated insulin secretion. Octreotide, a somatostatin analogue, is used in the treatment of hypoglycaemia in Diazoxide unresponsive CHI, but is associated with side effects such as gastrointestinal dysmotility and rarely, necrotising enterocolitis. It would be important to recognise rare but serious side effects from Octreotide therapy, particularly with long-term use. In this report, we have described drug-induced hepatitis with moderately high doses of Octreotide in a child with diffuse CHI. While serum alanine transaminase levels rose significantly with Octreotide therapy (maximum dose 30 μg/kg/day), hepatitis resolved following discontinuation of medical treatment. Liver enzymes should be monitored routinely in children with CHI using long-term Octreotide treatment, particularly with high doses. The presence of drug-induced hepatitis should prompt discontinuation of Octreotide treatment with likely subsequent resolution.