SUMMARY: Lodderomyces elongisporus infrequently causes bloodstream infections and has been isolated from Asia and Mexico. We encountered a catheter-related bloodstream infection, which involved some risk factors, due to L. elongisporus masquerading as Candida parapsilosis. A 39-year-old man who received a total arch and thoracoabdominal aortic replacement was admitted with a diagnosis of aorto-esophageal fistula. After thoracic drainage for the aorto-esophageal fistula, a catheter-related bloodstream infection was diagnosed. Micafungin (100 mg/day) was successfully administered to treat the catheter-related bloodstream infection for 42 days in total. The bloodstream and catheter tip yeast was grown on Candida agar medium and produced dark green colonies indicating Candida albicans. We performed sequencing analysis using a GenBank BLAST search. The sequence of the internal transcribed spacer region was 99.9z identical with that of the type strain L. elongisporus. This yeast organism has frequently been technically mistaken for non-albicans Candida spp. Furthermore, the prognosis and risk factors of L. elongisporus infection remain unclear owing to the scarcity of reported cases. Catheter-related bloodstream infection caused by this organism has not been described to date.