Invasive mold infections (IMIs) are significant causes of infectious mortality in immunocompromised patients, such as those with hematologic malignancies and allogeneic bone marrow transplant recipients. Of the IMIs, invasive aspergillosis is by far the most common. Nevertheless, over the past decade, other filamentous molds, such as Fusarium species, have been increasingly reported as the cause of severe IMIs in these patient populations. Herein we critically review the epidemiology, pathogenesis, clinical presentation, diagnostic methods, and therapeutic approaches for invasive fusariosis in immunocompromised hosts. We also present the key characteristics and differentiating features of invasive fusariosis and invasive aspergillosis. Current therapeutic approaches for fusariosis are suboptimal, resulting in exceedingly high mortality rates. At present, prompt diagnosis along with rapid initiation of appropriate treatment and, more importantly, reconstitution of the host immune responses are critical for a favorable outcome of this devastating opportunistic mycosis.