BACKGROUND: Cerebral aspergillosis, often encountered in immunocompromised patients, is almost always fatal despite radical surgical and medical management and frequently is a finding at autopsy. Attempts at fungal isolation often are unsuccessful, and a high index of radiologic suspicion is necessary. CASE DESCRIPTION: A premature, 5-month-old female infant, born via normal vaginal delivery, presented with a progressive increase in head size since birth, delayed developmental milestones, and intermittent vomiting for 1 month. There was no history of trauma and no evidence of diabetes mellitus or hypertension, family history of tuberculosis, gestational diabetes, jaundice, or febrile eruptions. Neonatal jaundice was present after day 1, remained for 2 days, and was treated with phototherapy. Findings of the general and systemic examinations were unremarkable. Findings of a neurologic examination revealed a hypoactive infant with papilledema and extensor plantar bilaterally. Computed tomography scan of brain showed hydrocephalus attributable to aqueductal stenosis. On endoscopic third ventriculostomy, dense, friable, whitish yellow, nonvascular masses were encountered in the third ventricle, obstructing it. Her endoscopic third ventriculostomy success score was 10 + 0 + 10 = 20%. Histopathologic examination and culture revealed Aspergillus flavus. A week later, a medium-pressure ventriculoperitoneal shunt was done, and voriconazole was added. RESULTS: At discharge on the 7th postoperative day, 4 weeks and 12 weeks later (ie, first and second follow-up) the child was active, playful, and feeding normally. The first reported case of an aqueductal stenosis due to aspergillosis was in 2000 by van Landeghem FK et al. (Clin Neuropathol 19:26-29, 2000). To the best of our knowledge, this may only be the second reported case of hydrocephalus attributable to aqueductal stenosis caused by Aspergillosis. In conclusion, aspergillosis should not be ruled out as a differential diagnosis in aqueductal stenosis, even when the patient seems to be immunocompetent.