Rhabdoid tumour is a rare childhood tumour with poor prognosis. We report a 13-month-old Malay girl suffering from this tumour that was located at the left fronto-temporo-parietal region of the brain. Computed tomography showed a large irregular enhancing mass that caused obstructive hydrocephalus. The tumour did not reduce in size after three operations and finally the patient succumbed to the disease four months after diagnosis.
Primary central nervous system (CNS) atypical teratoid/rhabdoid tumours (ATRT) are highly malignant neoplasms which usually present in infancy or early childhood. Although ATRT may arise anywhere within the CNS, the majority (approximately two-thirds) arise in the cerebellum or posterior fossa, and the remainder in the cerebrum. We described the imaging characteristics of CNS ATRT in the posterior cranial fossa of a 14-month-old boy.