A case of colonization of a prosthetic mitral valve in a 73-year-old Spanish male by the fungus Acremonium strictum W. Gams is described. The valve was replaced due to paravalvular leak and severe insufficiency and the patient died of multiorgan failure. The identity of the fungus was determined by morphological studies and it was confirmed by the analysis of the ITS region sequence analysis. Molecular studies seem to demonstrate that A. strictum is a species complex. The case emphasizes the potential high risk of fungal infection for patients with prosthetic valves.