Throughout the mountains of South America, Polylepis forests and the bird community they host stand out as one of the ecosystems of greatest conservation priority. These forests form one of the highest altitudinal treelines in the world and are centers of biological diversity and endemism. However, a variety of ecological characteristics and human pressures make them increasingly vulnerable to habitat degradation and future climate change. To guide conservation efforts, we reviewed the state of knowledge for bird species associated with Polylepis forests. We describe how the Polylepis forest ecosystem’s ability to support birds is influenced by its continental distribution and by local and landscape factors, including human activities and perturbations. We identified 29 priority areas for Polylepis conservation, which collectively support 44 endemic, threatened or highly specialized bird species associated with Polylepis forests. Of these, two are categorized as of critical situation, 8 as endangered, 8 as vulnerable and 9 as near threatened, according to the IUCN List of Threatened Species. In addition, 70% of these species (31) has restricted distribution ranges and are considered endemic to their country of origin, while 52% (23) has a high or medium degree of association to Polylepis forests. Recommendations from various studies agree that increasing patch connectivity and matrix permeability, and reducing soil erosion, burning and livestock stocking rates would contribute to improve the conservation and restoration of Polylepis ecosystems and the birds that inhabit it.