A 52-year-old male presented with a 25-year history of decreasing vision. Best corrected visual acuity was 0.3 in his right and 0.2 in his left eye. Fundoscopic examination showed bilateral symmetric atrophy of the retinal pigment epithelium and choriocapillaris in the posterior polar areas between vascular arcades and surrounding the optic disc. On fluorescein angiography, the large choroidal vessels beneath these affected regions were easily seen. Fundus autofluorescence imaging showed clearly defined hypoautofluorescent areas that corresponded to the aforementioned lesions. Atrophy of the choriocapillaris and outer retinal layer were detected in optical coherence tomography. Photopic and scotopic responses were subnormal in flash electroretinogram (ERG), and responses were also minimal in pattern ERG and multifocal ERG. The patient was diagnosed with posterior polar central choroidal dystrophy. We aimed to present the results of fluorescein angiography, fundus autofluorescence imaging, optical coherence tomography, and electrophysiological tests in this rare case of posterior polar central choroidal dystrophy.