A 58-year-old man with bilateral pigmented paravenous retinochoroidal atrophy (PPRCA) associated with macular coloboma in the right eye underwent color fundus photography and fundus autofluorescence with the California ultra-widefield retinal imaging system (Optos, Dunfermline, UK), spectral-domain optical coherence tomography (SD-OCT) (Heidelberg Spectralis HRA + OCT; Heidelberg Engineering, Heidelberg, Germany), and en face OCT angiography (OCTA) (AngioPlex, Cirrus HD-OCT 5000; Carl Zeiss Meditec, Dublin, CA). The patient presented with a visual acuity of counting fingers in the right eye and 20/32 in the left eye. Fundus examination and SD-OCT showed typical PPRCA alterations in both eyes and a macular coloboma in the right eye. The OCTA showed relative sparing of the retinal capillary plexuses, with diffuse defects in the choriocapillaris. The authors concluded OCTA imaging of PPRCA suggests more insights of the pathogenesis of this disease, showing that the disease primarily affects the choroidal vascular network, with a relative sparing of the retinal vasculature. © 2018 Slack Incorporated. All rights reserved.