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Objective: To report the successful outcome with a single intravitreal ranibizumab injection in a 15-year-old boy with Bietti crystalline dystrophy (BCD) who developed a unilateral macular neovascularization (MNV)Methods: A retrospective case reportResults: A 15-year-old Caucasian boy with Bietti crystalline dystrophy was diagnosed to have a unilateral MNV a year after the initial examition with the help of multimodal imaging and he was treated with a single intravitreal ranibizumab injection. Five months later, visual acuity improved and anatomically a dry-looking macula was noted.Conclusion: MNV is among the rare macular complications of BCD. The present case is the third reported pediatric case with MNV in association with BCD and the first pediatric BCD patient who received a vascular endothelial growth factor inhibitor (anti-VEGF) agent.