Until recently, Malassezia furfur was thought to be a pathogen only in tinea versicolor. More recently, this lipophilic yeast has been recovered from sick neonates with catheter-related infections. Malassezia fungemia was studied in seven patients, and the salient features of this infection in patients described in the literature were reviewed. Major risk factors include prolonged hospitalization, the presence of central venous catheters, and the use of intravenous fat emulsions. It is difficult to identify specific manifestations of fungemia in these complex cases occurring in patients with severe underlying disease; however, neonates often present with the signs and symptoms of sepsis and thrombocytopenia, whereas fever may be the only manifestation in adults. Some patients are asymptomatic. When symptoms are present, they resolve upon removal of the colonized catheter. The role of the lipophilic nature of Malassezia in the pathogenesis of infection is apparent from the ability of intravenous fat emulsions to support the growth of the fungus in vitro. A special solid medium that can be used to determine the true prevalence of malassezia fungemia has been devised. M. furfur must be considered in the differential diagnosis of opportunistic infections in patients receiving central hyperalimentation and should be sought by the culture of blood on appropriate medium.