PurposeTo assess the ability of swept source optical coherence tomography angiography (SS – OCT‐A) to identify abnormalities of the retinal and choroidal vascular network in macular dystrophies.MethodsOptical coherence tomography angiography and structural OCT were performed using a Swept‐source DRI OCT Triton (Topcon, Corporation, Japan) for patients with Stargardt disease, Best maculopathy, pseudo‐vitelliform dystrophy and progressive cone dystrophy. In our series, for every patient the clinical diagnosis was confirmed by the results of molecular genetic study.ResultsIn Stargardt patients, we found alterations especially located at the level of the deep capillary plexus and choriocapillaris. There were different degrees of choriocapillaris impairment, with no residual choriocapillaris inside area of retinal epithelium pigment atrophy in advanced stages. Large choroidal vessels and borders of the atrophy were well visualized. In patients with Best maculopathy and pseudo‐vitelliform dystrophy, OCT‐A revealed neovascularisation in the outer retina and the choriocapillaris while traditional multimodal imaging wasn't conclusive. In progressive cone dystrophy, there was a retinal vasculature signal reduction in both superficial and deep plexus associated with window effect in the outer retina and the choriocapillaris due to the retinal and RPE atrophy.ConclusionsIn inherited macular distrophies, OCT‐A may provide information of great interest. In stargardt disease, OCT‐A findings were in agreement with the results suggesting primitive severe involvement of choriocapillaris. In Best maculopathy and pseudo‐witelliform dystrophies, OCT‐A helps diagnose choroidal neovascularization. In Progressive cone dystrophies, it could be useful for gaining deeper Knowledge of both the pathogenesis and therapeutic implications.