Diffuse midline glioma (DMG) are distinct infiltrative subtype of glioma which is included as an separate entity in 2016 World Health Organisation Classification of Central Nervous System tumours. With the identification of H3K27M mutation which is the characteristic of DMG, a new horizon has opened up in Neurosciences for classification, identification and management of such tumours. Newer MRI sequences have immense scope in non-invasive diagnosis of such tumours. But still the caveat of whether to biopsy or not remains. With the advent of newer drug delivery system and targeted therapies with nanoparticles new hopes seems to be arising for patients with DMG. Although radiotherapy being the only approved therapy for DMG at present large multicentric trials and proper understanding of the biology and the natural course of the disease have shown a promising future for such dismal disease. We in this chapter would like to bring forth the pathogenesis, clinical feature, diagnostic and treatment modalities highlighting several trials for DMG.