PURPOSE: To report a rare case of bilateral giant retinal tear (GRT)-related retinal detachments in a 24-month-old male with Donnai-Barrow syndrome (DBS) and to review the current literature on this disorder. METHODS: Clinical information was obtained from the medical records of a patient with Donnai-Barrow syndrome seen at the Children's Hospital of Philadelphia. A literature review was conducted to identify all published cases of genetically or clinically diagnosed Donnai-Barrow syndrome. RESULTS: We report a case of a 24-month-old male with a history of DBS presenting with sudden loss of vision in both eyes. Ophthalmic examination revealed bilateral retinal detachments with GRT, complicated by grade C proliferative vitreoretinopathy (PVR) in the right eye. The patient underwent sequential scleral buckling, pars plana vitrectomy, membrane peeling, perfluoron, endolaser, fluid air exchange, and silicone oil placement in both eyes. Both eyes developed re-detachment, requiring repeat pars plana vitrectomy. Literature review revealed 10 reported cases of retinal detachments out of 38 DBS patients who survived past 1 year (26.3%). CONCLUSION: This case highlights the early presentation and complexity of GRT-related retinal detachment in DBS patients. Early, aggressive management using prophylactic laser retinopexy or scleral buckling may be essential in improving visual outcomes in DBS patients.