A new review of the clinical features, management and molecular aspects of BHD was recently published by Hasumi et al. (2015). The clinical features of BHD are discussed alongside the current management and treatment recommendations. In addition the review covers the identification of FLCN, FNIP1 and FNIP2 before summarising what is known about their functions based on animal models. Further understanding of FLCN function and the impact of its loss on tumourigenesis could enable the development of new treatments.