PURPOSE To describe a case of persistent placoid maculopathy in a patient with high myopia. METHODS Interventional case report. RESULTS A 59-year-old white man with high myopia presented with blurriness in the left eye. Vision was 20/20 bilaterally. Optical coherence tomography showed subretinal thickening and fluid without definitive leakage on fluorescein angiography after which anti-vascular endothelial growth factor (anti-VEGF) therapy was initiated. The patient was diagnosed with persistent placoid maculopathy based on multimodal imaging and clinical response. His disease course was characterized by multiple bilateral recurrences despite active treatment. At final visit (23 months after presentation), the vision was 20/20 in the right eye and 20/25 in the left eye, and the disease remained quiescent on immunomodulating therapy. CONCLUSION Prompt initiation of anti-VEGF injections and immunosuppression resulted in disease control and preservation of vision in both eyes for almost 2 years.