A 2-month-old infant who had undergone orthotopic liver transplantation at the age of 2 weeks for carbamoyl phosphate synthetase deficiency developed infection of the CNS due to Aspergillus fumigatus. The patient was successfully treated with administration of a combination of antifungal agents (including intraventricular amphotericin B), drainage of the parietal lobe abscess, and cessation of immunosuppression. An intraventricular catheter was used both to obtain ventricular fluid for microbiologic testing and to deliver amphotericin B during nearly 4 months of treatment. We review literature on aspergillosis in solid-organ transplant recipients, especially those in whom the disease involves the CNS, and discuss in particular clinical presentation, diagnosis, treatment, and outcome.