Candida infective endocarditis is a rare but serious entity that often requires aggressive treatment. However, treatment can be challenging in patients infected with drug-resistant fungi and/or with substantial comorbidity. Moreover, recommendations in treatment guidelines for these patients are based on limited clinical data due to their rarity. Here we report a case of Nakaseomyces glabrata (Candida glabrata) prosthetic valve endocarditis in a patient with congenital heart disease. This case illustrates a therapeutic dilemma for Nakaseomyces glabrata prosthetic valve endocarditis and the need for novel antifungal drugs and further clinical studies.