Abstract: A 56-year-old female presented with bilateral progressive blurred vision over 1 month. She has no known malignancy before her initial visit to our ophthalmologic clinic. Her best-corrected visual acuity decreased to hand motion from 30 cm in both eyes. Optical coherence tomography exhibited parafoveal thinning of outer retinal layers bilaterally. Fluorescein angiography and indocyanine green angiography disclosed hypofluorescent spots in late phase in both eyes. The suspicion of cancer-associated retinopathy (CAR) prompted us to investigate and refer for further systemic disease including occult malignancy. The patient was diagnosed with small-cell carcinoma of the endometrium or cervix, which is an extremely rare and aggressive neuroendocrine tumor. The patient was treated with oral prednisone with improved visual acuity. The patient expired from sepsis 2 months after her initial visit to our ophthalmologic clinic. In selected cases, CAR may present before the diagnosis of primary cancer. It is essential to recognize its ophthalmic manifestation for early discovery of primary malignancy.