Dyskeratosis congenita is a group of rare genetic bone marrow failure syndromes. 
Revesz syndrome, a variant disorder, is characterized by retinopathy, aplastic 
anemia, nail dystrophy, and cerebellar hypoplasia. We report the case of an 
11-month-old boy with bilateral cicatricial retinal detachments associated with 
fibrovascular proliferation. Genetic testing ultimately confirmed a diagnosis of 
Revesz syndrome, which can mimic cicatricial retinopathy of prematurity. Prompt 
referral to a hematologist expedites diagnosis and treatment.