Purpose <p>This is a description of the main middle cerebral artery (MCA) anomalies from the neuroradiological and embryological point of view, discussing the practical implications and the association with other vascular diseases.</p> Methods <p>Among the patients who underwent digital subtraction angiography (DSA) at our institution in a short time period (from 1 January 2021 to 31 December 2023), we selected three cases with high-quality images, representative for the main MCA anomalies, i.e., accessory, duplicated, and twig-like MCA.</p> Results <p>We describe a series of three cases, illustrating the three main embryological anomalies of MCA, including accessory MCA, duplicated MCA, and twig-like MCA. All patients underwent neuroradiological investigations at our institution because of the acute occurrence of neurological symptoms and the findings are presented using computed tomography angiography (CTA), magnetic resonance angiography (MRA), and DSA with a detailed description. These three variants have a very low prevalence and they were reported and described more systematically in the Asian population than in the European population. Both noninvasive and invasive neuroimaging techniques were used for investigating these patients, and catheter angiography still provides the more informative content for precisely imaging the angioarchitecture of these variants and the association with aneurysms on other cerebral arteries. The knowledge of the embryology of these variants is of paramount importance in order to make easier their identification and reporting and to enhance their understanding.</p> Conclusions <p>MCA anomalies should be known and carefully checked in order to avoid misdiagnosis with disease and may affect the treatment of acute stroke.</p>