Revesz syndrome is a variant of dyskeratosis congenita characterized by aplastic 
anemia, retinopathy, and central nervous system abnormalities. We describe a 
3-year-old boy in whom the spectrum of neuroimaging findings, including 
intracranial calcifications, cerebellar hypoplasia and unusual brain lesions 
were found by biopsy to be gliosis despite their enhancement and progression. In 
patients with dyskeratosis-related syndromes, non-neoplastic parenchymal brain 
lesions occur and gliosis should be considered in the differential diagnosis for 
progressive enhancing brain lesions. Should this finding be confirmed 
consistently in additional cases, brain biopsy could potentially be avoided.