We describe here the spontaneous remission of a diffuse brainstem lesion found in an infant at the age of 7 weeks. Clinical and MRI characteristics strongly suggested a diffuse pontine glioma. Repeated MRI studies showed a continuous decrease in the size of the lesion, which was no longer visible by the age of 27 months. Spontaneous remission of diffuse pontine glioma is extremely rare; to our knowledge there are reports of only three similar cases.