A case of systemic infection due to Saprochaete capitata in a patient with chronic lymphocytic leukemia is described. A review of the literature was conducted to identify all reported cases of this infection described between 1977 and August 2013. One hundred and four cases (included the present one) were identified. The median age of the patients was 56 years and 56% were males. Comorbidities included acute myeloid leukemia (52%), acute lymphoid leukemia (22%), other hematological malignancies (13%) and non-hematological diseases (9%). At the time of the infection, 82% of the patients were neutropenic. In 75% of the cases, the yeast was isolated from blood culture, in 25% from other sterile sites. Empirical treatment was done in 36% of the cases. Fifty-eight percent of the individual cases were treated with a combination or a sequential antifungal therapy. Amphotericin B was the antifungal drug most commonly used, followed by voriconazole and itraconazole. The overall crude mortality was 60%. Saprochaete capitata causes life-threatening infections in neutropenic patients. This comprehensive literature review may help the clinician to optimize the management of this rare infection.