Diffuse intrinsic pontine glioma (DIPG) is the most frequent brainstem tumor in children. All therapeutic efforts are palliative, being focal irradiation the only therapy capable of reducing temporarily tumor volume. The vast majority of patients succumb to their disease during the first 2 years after diagnosis usually due to local progression. At first relapse, focal re-irradiation has shown to provide clinical and radiologic benefits. There are no reports of craniospinal irradiation (CSI) as a rescue regimen when progression is local and disseminated. We report a patient treated with CSI at metastatic progression showing clinical and radiological response with good tolerance.