The fungus Paecilomyces lilacinus is an emerging pathogen that causes severe human infections, including devastating oculomycosis. Usually, it shows low susceptibility to conventional antifungal drugs in vitro, and variable susceptibility to novel triazoles. A review of the published literature identified 119 reported cases of human infection by P. lilacinus between 1964 and 2004. Most were cases of oculomycosis (51.3%), followed by cutaneous and sub-cutaneous infections (35.3%), and a smaller group of miscellaneous infections (13.4%). Lens implantation is the most frequent predisposing factor for oculomycosis. Cutaneous and sub-cutaneous infections occur mainly in solid organ and bone marrow transplant recipients, although surgery and primary or acquired immunodeficiency are also relevant predisposing factors. Infections in apparently immunocompetent patients have also been reported. Surgical debridement combined with antifungal drug therapy, or the correction of predisposing factors, such as neutropenia, are usually required to obtain improvement. Treatment with traditional antifungal drugs often fails. Voriconazole has demonstrated good activity in both cutaneous and ocular infections in the few cases in which this drug has been used. The new triazoles ravuconazole and posaconazole show good in-vitro activity against P. lilacinus and could be promising therapeutic alternatives.