Pichia fabianii, a yeast rarely causing human infections, was isolated from the blood of a patient with aortic valve endocarditis. The isolates were initially identified biochemically as Candida pelliculosa, but based on direct sequencing of the ITS2 region of rRNA, they were subsequently reidentified as P. fabianii. Antifungal therapy with fluconazole and later with voriconazole led to the development of resistant variants which had high MIC values to both antifungals. Strong biofilm formation by this yeast could also have played a role in the development of its resistance and allowed for its persistence on the infected valve during antifungal therapy. To our knowledge, this is the first published case of endocarditis and the fourth human infection caused by this yeast species.