Background: The importance of fungal infections has risen because of progress in medicine. Normal host is susceptible to fungal infections, albeit to a smaller  spectrum of organisms.  Fungal diseases involving the brain are usually secondary to systemic or pulmonary infection, and, in most patients, spread hematogeneously.   Little is known about CNS aspergillosis in children and whether there are any differences from adult. Novel agents such as amphotericin B lipid formulations, newer azoles and echinocandins have entered the armamentarium of antifungal drugs, but invasive aspergillosis (IA) such as CNS aspergillosis remains to be a devastating opportunistic infection and a  medical challenge with a published mortality rate of >80%.

Objective: This report aims to provide the readers increase awareness on clinical presentation, evaluation, and early and accurate diagnosis of neuroaspergillosis in young patients.

Case: We present a case of a 4 year old female with signs of increase intracranial pressure. Cranial MRI with contrast showed non-enhancing posterior fossa cystic masses with obstructive hydrocephalus. The patient underwent operation and the histopathological diagnosis showed fungal elements. Serum Aspergillus Galactomannan antigen assay is positive at 0.724 (cut off: <0.5 normal). The patient was given Amphotericin and later Voriconazole.

Conclusion: This case highlights the importance of early recognition and high index of suspicion of CNS aspergillosis in a young patient presenting with increase intracranial pressure. Search for more appropriate therapy may improve the outcome of this life threatening infection.