We describe MR findings of a nasal glioma in a 5-week-old male infant with feeding difficulties and symptoms of respiratory distress. Endoscopic examination revealed a soft tissue mass in the nasal cavity. In T1- and T2-weighted images, the gyral structure of grey matter was visible. In T1-weighted images, a tissue component with a lower signal intensity equal to that of white matter was also well distinguishable. Edge enhancement of the tumour was due to the nasal mucosal epithelium covering the tumour. MR images were useful to rule out intracranial extension of the nasal cavity lesion and the brain, thus excluding the diagnosis of encephalocele. The lesion was excised and histologically characterized as heterotopic brain tissue.