Carotid cavernous fistulas (CCFs) are rare but clinically significant vascular anomalies characterized by abnormal connections between the cavernous sinus and arteries. This overview presents acomprehensive analysis of anatomy, classification, clinical presentation, diagnosis, imaging, and therapy of CCFs. The cavernous sinus, acentral venous structure in the brain, is of critical importance for understanding CCFs due to its proximity to key structures such as the internal carotid artery and cranial nerves. CCFs are classified into direct and dural types, with direct fistulas typically being high-flow and dural fistulas being low-flow. The symptomatology varies greatly and can range from noises in the head, diplopia, red eye, tearing, to blurred vision and headaches. The diagnostic assessment requires acombination of detailed medical history, neurological and ophthalmological examination, and the use of imaging techniques. In imaging, computed tomography (CT) and magnetic resonance imaging (MRI) are crucial for depicting the anatomical structures and blood vessels, while digital subtraction angiography (DSA) is considered the gold standard for accurate representation of the fistula. The treatment of CCFs is complex and depends on the type of fistula, location, and clinical condition of the patient. This overview emphasizes the importance of precise diagnosis and individualized therapy to achieve optimal results and avoid complications. Ongoing developments in medical imaging and treatment techniques will continuously improve the treatment outcomes of patients with CCFs.