Enteric neuropathies comprise a vast and disparate array of congenital and acquired disorders of the enteric nervous system (ENS), reflecting both the complexity of its neuronal composition and the many interactions that modulate its function. Although present therapeutic strategies, largely limited to surgery and the provision of artificial nutrition, have transformed the early survival and life of sufferers, levels of morbidity and mortality remain unacceptably high. This highlights the need to develop new treatments for enteric neuropathies. In the last decade, the tremendous advances in molecular biology and genetics have significantly enhanced our understanding of ENS development and function. Coupled with equivalent progress in the fields of pharmacology and stem-cell biology, this has led to the identification of novel tools and targets for therapy, which either aim to optimise the function of the intrinsic ENS or replace/replenish components of an inadequate or dysfunctional ENS. This article reviews current work on a number of these interventions with a particular focus on the use of ENS stem cells as potential therapeutic tools for enteric neuropathies.