Diffuse leptomeningeal glioneuronal tumours (DL-GNT) are rare, with an unknown incidence but fewer than 100 cases reported since 2012. The clinical presentation is non-specific, ranging from abdominal to neurological symptoms. Presently, definitive radiological criteria aren’t established, but some features, such as nodules, characteristic extension patterns and post-contrast leptomeningeal enhancement, are found to be prominent. We present the case of a 14-year-old male with an advanced case of DL-GNT, with MRI showing all the features of what is currently thought to be the typical radiological presentation. The patient is currently undergoing treatment but remains severely handicapped by the disease.