Less frequent retinal disorders associated with vascular alterations frequently present a diagnostic and therapeutic challenge. Vascular alterations may develop either at the posterior pole or peripheral of the vascular arcades, while subjective signs and visual function loss usually develop when the macula is involved. The differential diagnosis includes myopic macular neovascularisation, macular telangiectasia type 1, MacTel 2, pseudoxanthoma elasticum with angioid streaks, retinal arterial macroaneurysms, Coats disease and the combination of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN syndrome). Due to the low prevalence, early signs are often overlooked or misdiagnosed, and the diagnosis is established when secondary complications develop. Early detection is of high relevance for the affected patients: treatment in early stages may avert severe and permanent visual function loss. The development of multimodal retinal imaging techniques including optical coherence tomography, optical coherence tomography angiography, and widefield fluorescein angiography has markedly improved the early detection and differential diagnosis of these disorders. Differential diagnosis and definition of the disease stage is mandatory for a treatment according to current guidelines, recommendations or clinical experience. Larger studies to define optimal treatment regimens require multicenter cooperation and are not available for all disorders mentioned. The present review provides a concise overview of the early diagnosis, differential diagnosis and stage-related treatment regimens of these less frequent retinal vascular disorders.