Acrodermatitis enteropathica (AE) is a rare congenital disorder owing to an abnormality with intestinal absorption and/or transportation of zinc. We describe two male siblings, who presented with evidence of both acute and chronic zinc deficiency, despite being diagnosed with AE very early in life. We wish to highlight the importance of sustained high-dose zinc supplementation and regular monitoring in AE cases. Proper counselling of parents about the need for life long supplementation and increasing requirements with age is essential.