OBJECTIVE To describe a unique case of cystoid macular edema associated with Ibrutinib treatment for Chronic Lymphocytic Leukemia (CLL). METHODS A 73-year-old male patient presented to the ophthalmology clinic complaining of decreased vision in his seeing-eye ('only eye', left). Further clinal examination and imaging revealed the presence of a cystoid macular edema (CME). With no apparent cause to this condition, topical treatment with NSAIDS and steroids continued over two years with only partial response and persistent macular edema, resulting in decreased vision. Cessation of Ibrutinib treatment resulted in resolution of the macular edema and improvement in visual acuity over 6 months. CONCLUSIONS Several novel oncologic therapies have been associated with CME in recent years. This case demonstrates an association between Ibrutinib an oral, irreversible inhibitor of Bruton's Tyrosine Kinase (BTK), and the development of CME. CME was resistant to topical treatment but resolved after treatment cessation. Along with two previous cases reported, this case suggests that CME is a rare adverse event of Ibrutinib therapy. Screening for CME in Ibrutinib treated patients who report visual symptoms should be considered.