Congenital glioblastomas are rare primary tumours of the central nervous system with poor prognosis if left untreated. We report the case of a 4-week-old infant with such a tumour treated by surgical excision and a course of postoperative chemotherapy. After the chemotherapy, the patient displayed a neurological deterioration and follow-up magnetic resonance imaging (MRI) scans revealed no signs of recurrence, but showed entrapment and significant distension of the right lateral ventricle. A novel technique of contralateral cystoventriculostomy was performed to attempt to decompress the cyst and improve the infant's neurological status. After surgery there was a significant neurological improvement, and 30 months after resection the child is alive, with mild hemiparesis and no signs of recurrence on follow-up MRI scan. The cystoventriculostomy is patent and continues to decompress the encapsulated ventricle.

Preoperative MRI has both good sensitivity and positive predictive value. Specificity and negative predicitve value were limited in this series. No correlation was found between preoperative imaging and outcome. Both radiological and clinical criteria are important for patient selection.