In this first-of-its-kind report, we describe an unusual case of a 32-year-old man who harboured two biologically distinct metachronous brain tumours. He presented with features of raised intracranial pressure 8 years after total excision of a right temporal ganglioglioma. Imaging showed a heterogenously contrast enhancing bifrontal lesion with ventricular extension. Histopathological examination revealed an anaplastic ependymoma. While a co-incidental occurrence is a possibility, there could be possible mechanisms to explain this pathological oddity.