Introduction: A 49-year-old woman presented at Inoda Eye Clinic with inferior visual field defect in the left eye. Ophthalmoscopic examination revealed well-demarcated retinal edema of the superior region at the posterior pole in the left eye. The area of retinal edema was consistent with the area of the cilioretinal artery, and therefore, cilioretinal artery occlusion was suspected. Optical coherence tomography showed hyperreflective bandlike lesions involving the middle layers of the retina at the level of the inner nuclear layer. Optical coherence tomography angiography showed decreased flow sign in outer retina layer and choriocapillaris layer rather than in superficial layer. Based on these findings, the patient was diagnosed with paracentral acute middle maculopathy due to cilioretinal artery occlusion. Optical coherence tomography angiography is a useful diagnostic examination instead of fluorescein angiography for evaluation of cilioretinal artery occlusion.