Diffuse intrinsic pontine glioma (DIPG), now reclassified as diffuse midline glioma (DMG), is the most common cause of mortality from paediatric central nervous system (CNS) tumours. Diagnosis is made based on characteristic clinical presentation and neuro-imaging findings. Prognosis is poor, with minimal therapeutic options, reflected in a median survival of under 12 months. We present a patient with Pendred syndrome, diagnosed with DMG at 2-years of age with characteristic presentation and neuro-imaging findings, who remains asymptomatic and well at nearly 4-years post diagnosis despite progression of the primary lesion on serial imaging.