Hereditary angio-oedema is an uncommon condition, which usually first presents in childhood. In the absence of appropriate treatment hereditary angio-oedema has a high mortality. There are a number of reports of successful short- and long-term treatment with individual agents in those who suffer from hereditary angio-oedema. However, there is little guidance in the literature for the overall management of children and adolescents with this condition. Here we review the treatment options available for children and adolescents with hereditary angio-oedema, and propose a simple management guideline for such patients.