A 15-year-old female child presented with sudden and painless vision loss in the left eye. Visual acuity was 1,0 in the right eye and 0.8 in the left eye from the inferior half of the visual field. Anterior segment examination was normal. The fundus examination of the left eye revealed retinal whitening in the superior temporal and superior nasal of the the optic disc, the superior part of macula, the inferior half of macula and the inferior half of the retina, and segmentation in the blood column in the inferior branches of the retinal artery was observed, while the right eye was normal. On optical coherence tomography (OCT), diffuse thickening and hyperreflectivity in the superficial layers and shadowing in the outer layers of the retina were observed in the inferior macula and retina in the left eye. In the superior part of the macula, OCT revealed a characteristic hyperreflective band-like lesion on the inner nuclear layer consistent with PAMM. The patient was diagnosed with left inferior hemiretinal artery occlusion and her treatment was planned. The systemical examination of the patient revealed mitral valve stenosis and insufficiency.

When retinal artery occlusion is seen in the pediatric age group, a comprehensive etiological investigation is required. PAMM may be associated with BRAO due to mitral valve disease in a pediatric case. Keywords : branch retinal artery occlusion, mitral valve disease, paracentral acute middle maculopathy, pediatric age group