A baby boy who had a left facial mass detected on antenatal ultrasound was delivered by Caesarian section after foetal distress was detected. Imaging investigations by plain radiographs and MRI showed a large mass with calcifications, soft tissue, fat and fluid components. A total surgical excision was perfomed and histology examination showed teratoma with no malignant features. Two weeks postoperatively, there was rapid recurrence of the tumour with intracranial involvement and obstructive hydrocephalus shown on MRI. The tumour was inoperable at surgery and the baby subsequently died at 5 weeks of life. This case describes the clinical course and imaging features of a neonatal epignathus teratoma with malignant and aggressive features.