Background: Candida meningitis/ventriculitis is rather rare during childhood. In this study, we investigated the clinical characteristics, risk factors, treatment, and prognosis of patients with Candida meningitis/ventriculitis.Methods: Patients under the age of 18 years who were diagnosed with Candida meningitis/ventriculitis were evaluated retrospectively.Results: A total of 10 cases with Candida meningitis/ventriculitis were analyzed. Three patients (30%) were below the age of one, and two (20%) were neonates. The two most common underlying conditions were hydrocephalus shunt and prematurity. Predisposing factors were a history of broad-spectrum antibiotic use, external ventricular drainage, total parenteral nutrition, central venous catheter, and staying in intensive care. The cerebrospinal fluid culture was positive in all patients, and 10% (1/1) had bacteremia. Of the isolates, 50% were C. albicans, 30% were C. tropicalis, 10% were C. lusitaniae, and 10% were C. dubliniensis. Fluconazole treatment was initiated in four (40%) and voriconazole in three (30%) patients. Two patients received combined treatment (amphotericin B + fluconazole/voriconazole). The median treatment duration was 38.6 days (range: 16–70 days). Three patients received intraventricular Amphotericin B.Central nervous system devices which were assumed to be infected were removed. A complication of endophthalmitis developed in one patient. The mortality rate was 10%.Conclusions: Among agents causing meningitis/ventriculitis, Candida should also be kept in mind in premature neonates and patients with ventricular-peritoneal shunts. The history of antibiotic use and external ventricular drainage are important predisposing factors. It can be successfully treated with fluconazole, voriconazole, amphotericin B, and removal of the central nervous system device.