PURPOSE: To report a patient who developed cancer associated retinopathy (CAR) in the setting of metastatic gastrointestinal stromal tumor (GIST) of stomach. OBSERVATIONS: A 70-year-old man with the history of GIST presented to a tertiary eye center with blurry vision and gradually progressive nyctalopia in both eyes. Visual acuity (VA) was 20/40 in both eyes. Detailed investigations revealed progressive generalized constriction of visual fields with central scotomas and enlarged blind spots in both eyes, symmetrically reduced scotopic and photopic amplitudes on full field ERG, and positive antiretinal antibodies against CONCLUSION AND IMPORTANCE: Although CAR is most seen in patients with carcinoma, the index case highlights that CAR may also be seen in a patient with GIST. Local therapy with intravitreal dexamethasone implant as the first line management may be supportive of a diagnosis of CAR, as well as useful given its rapid local effect while waiting for systemic assessment and therapy.