Brainstem gliomas, particularly diffuse intrinsic pontine gliomas (DIPG), continue to present formidable challenges in neuro-oncology. Diagnosing brainstem gliomas hinges on a combination of clinical evaluation, neuroimaging, and molecular profiling. Advanced magnetic resonance imaging (MRI) techniques, such as functional MRI and spectroscopy, facilitate precise localisation and characterisation. Tumour biopsies remain controversial due to the inherent risks associated with brainstem surgery but may offer prognostication and treatment tailoring based on H3 K27M mutation status. Surgical resection, though traditionally not considered an option, may still be explored in dorsal low-grade exophytic lesions (some of which may turn out to be piloctic astrocytomas) and may offer long-term control in these patients. Radiotherapy, either adjuvant or radical, is the treatment standard at present. Benefit of systemic agents such as temozolomide has not been seen in brainstem gliomas. Overall prognosis is dismal except for a small subset of patients. Novel target and treatments need to be explored to improve control.