Epidermolysis bullosa (EB) encompasses a group of inherited blistering skin disorders classified into three main subtypes of simplex, junctional and dystrophic. In recent years there have been substantial advances in our understanding of the molecular basis of these conditions and in the management of such patients. In spite of this progress, squamous cell carcinoma (SCC) is still a major cause of morbidity and mortality, particularly in Hallopeau--Siemens recessive dystrophic EB. The reason why dystrophic EB patients readily develop SCC with such a poor prognosis remains a mystery. This article reviews the epidemiology of cancer in inherited EB and also discusses the clinical features, histological assessment and treatment options of SCC in EB.