The changes in the cardiovascular system are associated with ocular manifestations, often as a consequence of pathological alteration in the ocular vasculature. The ease of visualization of these retinal changes makes the eye a window to the cardiovascular system. Certain congenital cardiac defects lead to changes in the retinal vascularity due to increased tortuosity and dilatation. In adults, the arterial dissection of internal carotid and vertebral arteries present as amaurosis fugax with or without oculo-sympathetic palsy. The patients with untreated infective endocarditis present with Roth spots, retinitis, embolic retinopathy, or sub-retinal abscesses. Hypoperfusive, hypertensive, or “mixed” retinopathy is a hallmark sign in patients of untreated infective endocarditis. Giant cell arteritis can present with ischemic ocular symptoms that may precipitate in irreversible vision loss. Systemic vascular manifestations such as coronary artery disease may manifest in a wide range of symptoms from amaurosis fugax to vision loss depending upon the size and location of retinal emboli. Rare cardio-oncological pathologies such as myxomas result in vision loss secondary to central retinal artery occlusion. A high clinical suspicion in patients with history of cardiovascular diseases can help in early diagnosis and management of impending, adverse cardiovascular and cerebrovascular events. In this review, we comprehensively discuss the spectrum of cardiac and vascular diseases with ocular manifestations as well as highlight the typical ocular presentations associated with these pathologies.