Aims To discuss foveal development in the context of detailed retinal vasculature imaging in foveal hypoplasia using optical coherence tomography angiography.

Methods In this case series, the optical coherence tomography angiography results of four patients with idiopathic foveal hypoplasia and two patients with foveal hypoplasia secondary to oculocutaneous albinism are presented.

Results Cases with intact visual acuity demonstrated lower grades of foveal hypoplasia on optical coherence tomography, while those with poor vision demonstrated high grades of foveal hypoplasia. The superficial retinal capillary plexus was intact in the foveal area in all cases, with no demonstrable foveal avascular zone. The deep retinal capillary plexus was absent to variable degrees in most cases, but was most persistent in those cases with reduced vision.

Conclusions The superficial retinal capillary plexus is present in cases with foveal hypoplasia, while the deep retinal capillary plexus is absent to varying degrees. Our findings support the hypothesis that an intact foveal avascular zone of the deep capillary plexus allows for outer retinal photoreceptor specialisation to occur unimpeded, resulting in preserved visual acuity, while this process may be inhibited by an absent deep capillary foveal avascular zone with resultant poor vision.