Classic galactosaemia occurs due to GALT deficiency and is associated with jaundice, weight loss, cataract formation, mental retardation and cirrhosis due to excessive accumulation of galactose in the respective tissues in late infancy or early childhood.2 Patients with galactokinase deficiency have jaundice only and mental retardation is not a characteristic feature in these children.1 Neonatal screening for galactosaemia is not routine in the Indian subcontinent. The gene discussed is GALK1; the disease is hyperinsulinemic hypoglycemia, familial, 4.