Uncommon, or rare, yeast infections are on the rise given increasing numbers of immunocompromised and seriously-ill patients. The major pathogens include those of the genera Geotrichum, Saprochaete/Magnusiomyces and Trichosporon species as well as Kodamaea, Malassezia, Pseudozyma (Moesziomyces/Dirkmeia), Rhodotorula, Saccharomyces and Sporobolomyces species. A considered approach to the complex, multidisciplinary management of infections caused by these pathogens is essential to optimising patient outcomes; however current management guidelines are either region-specific or require updating. In alignment with the “One World–One Guideline” initiative to incorporate regional differences, experts from diverse geographic regions analysed the published literature describing the epidemiology and management of the above rare yeasts. This guideline summarizes the consensus recommendations with regards to the diagnostic and therapeutic options for these rare yeast infections with the intent of providing practical assistance in in clinical decision making. Because there is less clinical experience of rare yeast infections and studies on these were not randomized nor were groups compared, most recommendations are not robust in their validation but represent insights using expert opinions and in-vitro susceptibility results. We report in the present manuscript the key features of the epidemiology, diagnosis, antifungal susceptibility and treatment outcomes of Geotrichum, Saprochaete/Magnusiomyces and Trichosporon infections.