Background: Candida haemulonii complex-related species are pathogenic yeasts closely related to Candida auris with intrinsic antifungal resistance, but few epidemiological data are available. Methodology/Principal findings: We analyzed clinical and demographic characteristics of patients with fungemia due to C. haemulonii complex and related species (C. pseudohaemulonii, C. vulturna) reported in France during 2002–2021, and compared them to data of C. parapsilosis fungemia, as they all can be commensal of the skin. We also conducted a study on adult inpatients and outpatients colonized by C. haemulonii complex, managed at the University Hospital of Martinique during 2014–2020. Finally, we performed a literature review of fungemia due to C. haemulonii complex and related species reported in Medline (1962–2022). In total, we identified 28 fungemia due to C. haemulonii complex in France. These episodes were frequently associated with bacterial infection (38%) and high mortality rate (44%), and differed from C. parapsilosis fungemia by their tropical origin, mainly from Caribbean and Latin America. All isolates showed decreased in vitro susceptibility to amphotericin B and fluconazole. In Martinique, we found that skin colonization was frequent in the community population, while colonization was strongly associated with the presence of foreign devices in ICU patients. The literature review identified 274 fungemia episodes, of which 56 were individually described. As in our national series, published cases originated mainly from tropical regions and exhibited high crude mortality. Conclusions/Significance: Multidrug-resistant C. haemulonii complex-related species are responsible for fungemia and colonization in community and hospital settings, especially in tropical regions, warranting closer epidemiological surveillance to prevent a potential C. auris-like threat. Author summary: Yeasts of the C. haemulonii complex (C. haemulonii sensu stricto, C. duobushaemulonii, C. haemulonii var. vulnera) and related species (C. pseudohaemulonii and C. vulturna), are phylogenetically close to Candida auris, but their distribution, pathophysiology and antifungal resistance profiles are poorly known. This work provides the first epidemiological data on these yeasts in France. Fungemia caused by these yeasts were mainly identified in tropical overseas regions (French West Indies, French Guiana), and occurred in patients with risk factors for candidemia, particularly a cutaneous portal of entry. This work highlights the skin carriage of these yeasts in these tropical regions, and their ability to colonize foreign devices. Like for Candida parapsilosis, catheters are the main pathway for fungemia but mortality seems higher with yeasts of the C. haemulonii complex and related. They commonly show in vitro resistance to many antifungal agents, notably fluconazole and amphotericin B, which are still frequently used as first and second-line treatments for candidemia worldwide. The findings from the literature review are consistent with these overall results. These observations justify closer epidemiological surveillance in the concerned regions to prevent a potential C. auris-like threat. [ABSTRACT FROM AUTHOR]