The purpose of this case report is to present the first case of neratinib maculopathy. We describe the initial presentation, baseline characteristics, imaging findings, and outcomes. The case report is accompanied by a thorough literature review including possible mechanisms of tyrosine kinase inhibitor (TKI) maculopathy. Neratinib is a novel TKI that is commonly used in the treatment of breast-associated malignancies. Neratinib toxicity presents similarly to macular telangiectasia type II but differs with the fine granular hypofluorescent areas spanning the limit of the posterior pole and vascular arcades as well as the nasal aspect of the optic nerve. We report a case of suspected macular toxicity secondary to neratinib. Concomitant use of neratinib in conjunction with docetaxel and other chemotherapeutics with known retinal side effects should alert clinicians of an increase in the risk of macular toxicity. Albeit commonly reported ocular side effects of TKIs, maculopathy is a rare and potentially overlooked side effect. Patients that have planned chemotherapy should undergo a baseline retinal examination.