The authors present four children with acute comitant esotropia (AACE) associated with diffuse intrinsic pontine glioma. All were treated with radiation therapy and all subsequently died due to the tumour. Children were aged 5, 5, 4.5 and 11 years. General visual acuity was good on presentation, and all presented with comitant strabismus. One had a later development of bilateral 6th nerve palsy. Only one child had papilledema on presentation. Survival ranged from less than one year to four years. AACE due to neurological pathology is rare and thus neuro imaging is commonly delayed. The authors suggest this should be done if there are any unusual or atypical findings or with late presentation without significant refractive error, or variable angle of deviation. A high index of suspicion is important to aid differential diagnosis.