Rhinocerebral aspergillosis (RA) is becoming increasingly common in patients undergoing bone marrow transplantation (BMT). The disease can involve nearly all major head and neck structures, including the nose, paranasal sinuses, and orbits. Intracranial extension of the infection is of major concern, since this is usually a fatal complication. Our study population comprised 423 consecutive BMT patients at Hadassah University Hospital from January 1986 to August 1992. Eight patients (1.9%) developed RA, 5 of whom had underlying hematologic malignancies, and 3 of whom had severe aplastic anemia. Only 2 of the 8 patients responded completely to therapy, with a follow-up of 15 months. It appears that RA is a fatal complication in immunocompromised patients post-BMT. Early diagnosis followed by extensive surgical debridement of necrotic tissue and systemic, as well as topical, antifungal therapy with amphotericin B or its new formulations and the patient's recovery of bone marrow function may improve the outcome of this life-threatening complication.