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Purpose: To report the course of atypical choroidal neovascularization (CNV) in a middle-aged woman experiencing sudden vision loss., Observations: A middle-aged female presented with sudden onset vision loss. Following in depth investigations an initial diagnosis of presumed idiopathic CNV was made in her right eye. Multimodal imaging confirmed the presence of CNV, while the left eye remained unaffected. The CNV initially responded well to intravitreal Aflibercept injections, resulting in significant visual improvement. However, the patient later developed punctate inner choroidopathy (PIC), which eventually responded to systemic but not local corticosteroids. Recurrent CNV became incompletely controlled by Aflibercept. Switching to Faricimab resulted in sustained visual acuity improvement and CNV resolution., Conclusions and Importance: The therapeutic efficacy of anti-VEGF treatments has long been underscored in the management of CNV. However, CNV in younger patients often points to a different causation and sometimes requires different management. Our case underscores the significance of inflammatory causes of CNV and including anti-inflammatories in the treatment of CNV management and outlines differences in anti-VEGF efficacy in control of CNV., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors. Published by Elsevier Inc.)