Many of the clinical features associated with familial exudative vitreoretinopathy (FEVR) overlap with those commonly found in retinopathy of prematurity (ROP). Differentiating these two distinct entities may be challenging in premature infants. FEVR can be distinguished from ROP in that the former demonstrates exudates on examination and tends to recur or reactivate. The authors report the case of a premature infant diagnosed with FEVR and treated with diode laser who was subsequently lost to follow-up. Upon re-establishing care 3 years later, the patient demonstrated renewed bilateral vitreoretinopathy. This manuscript underscores the importance of accurate distinction between FEVR and ROP for the purpose of optimal patient management.