The monotypic Notiomys is the most poorly-known genus of Abrotrichini, a recently erected tribe of South American sigmodontine rodents. Based on a large sample, mostly recovered from owl pellets but including trapped individuals, we refine the known geographic distribution of this mouse ( providing 20 new contemporary recording localities and four fossil occurrences), relate this to the principal Patagonian environmental units, add new data on the type locality of the species, and comment on its conservation status. In addition, we present a detailed external and craniodental description and provide data showing geographic variation of some skull features. Finally, we provide preliminary data on genetic variation ( cytochrome b gene sequences) that demonstrate the existence of a low degree of observed differentiation (ca. 0.7%) between the populations analyzed.