Objectives The identification, drug resistence of and clinical significance of a rare fungus, Lodderomyces elongisporus (L. elongisporus), isolated from dialysate were investigated to improve the recognition and strengthen the importance of the infection of this fungus. Methods The culture and microscopic characteristics of the isolate was observed, comparing with 5 common yeasts (Candida albicans, Candida tropicalis,Candida parapsilosis, Candida glabrata, Issatchenkia orientalis). VITEK 2, matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS) and sequencing analysis were used to identify the isolated strain. Subsequently, MSP dendrogram was performed to investigate the affinity. In addition, minimum inhibitory concentration (MIC) of antimicrobial was determined by microdilution method. Results The pathogen, L. elongisporus, displayed white smooth colonies on the blood and PDA plate after culturing 24-96 h, but no color performed on CHROMagar medium on 24 h, and gradually appered a turquoise-blue colony at 48-96 hours. The VITEK 2 system identified it as Candida famata, however, MALDI-TOF analysis indicated a likely identification of L. elongisporus (score value=2.10). The sequencing analysis was applied using a GenBank BLAST search and the sequence of the internal transcribed spacer region (ITS) was 99.47% identical with that of the type strain L. elongisporus.The MSP dendrogram showed that L. elongisporus was closest to C. parapsilosis. Antibiotic susceptibility test (YeastONE) indicated the MIC of micafungin，caspofungin，5-fluorocytosine，posaconazole，voriconazole，itraconazole，anidulafungin，fluconazole and amphotericin B were0.015μg/mL、0.03μg/mL、0.12μg/mL、0.03μg/mL、0.03μg/mL、0.12μg/mL、0.03μg/mL、0.5μg/mL、0.25μg/mL, respectively. Conclusions L. elongisporus infection may have been underestimated due to the low isolation and few case reports. Laboratories and clinicians should pay more attention to L. elongisporus infection.