A retrospective study of 76 episodes of candidemia in 73 patients with underlying hematological malignancy, from 1988 until 1997, has been conducted to evaluate the clinical characteristics and to ascertain the variables related to the onset and the outcome of candidemia. The most frequent malignancy was acute myeloid leukemia (29 episodes). Candidemia developed mainly during aplasia in patients refractory to chemotherapy (42%). In 65 episodes (86%) the patients were neutropenic (ANC < 1×10⁹/l) before the candidemia diagnosis for a median time of 13 d, and in 53 episodes (70%) at microbiological diagnosis of candidemia ANC was <1×10⁹/l. Candida albicans was the most frequently isolated etiologic agent (31 episodes), but C. non‐albicans species sustained the majority of candidemia. Seventeen candidemias developed during azoles prophylaxis. One month after the diagnosis of candidemia, 26 patients died. In 19 cases, death was attributable to candidemia. The case‐control study demonstrated, at univariate analysis, that the colonization with Candida, spp. (p=0.004), antimycotic prophylaxis (p=0.01), presence of central venous catheter (p=0.01), neutropenia (p=0.002), and the use of glycopeptide (p=0.0001) increased the risk of candidemia. Using multivariate regression analysis only colonization with Candida spp. and the previous therapy with glycopeptide were associated with a significantly increased risk. Acute mortality, expressed by a cumulative probability of survival at 30 d from diagnosis of candidemia, was 0.67 (95% C.I. 0.55–0.77) and was significantly reduced in patients with neutrophils <1×10⁹/l when compared to those with neutrophils > 1×10⁹/l (p at Mantel‐Cox=0.029). Overall cumulative probability of survival at 1 yr was 0.38 (95% C.I. 0.27–0.49) and only the treatment with Amfotericin B significantly reduced the risk of death.