This report aims to describe the clinical presentation of a patient with an atypical central retinal vein occlusion (CRVO) in the setting of multiple myeloma. The patient was a 72-year-old man with an extensive hematologic-oncologic history and cardiovascular risk factors who presented with new-onset blurry vision and visual acuity (VA) of 20/40 in the left eye. The VA worsened to 20/60 two months after the initial presentation. By the 6-month follow-up visit, a series of three intravitreal bevacizumab injections for macular edema improved VA from 20/60 to 20/40. This case demonstrates the importance of fluorescein angiography in diagnosing a CRVO and exemplifies an atypical presentation on exam and imaging studies. [Ophthalmic Surg Lasers Imaging Retina 2024;55:730-733.].