There are several anomalies of the middle cerebral artery (MCA) in humans, such as accessory MCA, duplicated MCA, fenestration of MCA, and duplicated origin of MCA. Recently, unfused or twig-like MCA, which indicates MCA trunk occlusion with collateral plexiform arterial network, have been reported. During the embryonic stage, MCA is thought to generate from plexiform arterial twigs arising from the anterior cerebral artery, and these twigs form the definitive MCA by fusion and regression at the end of the development stage. Any interruption during the fusion of the arterial twigs may result in MCA anomalies, and the unfused or twig-like MCA, especially, is hypothesized to be the persistent primitive arterial twigs. Clinically, it is challenging to differentiate the unfused or twig-like MCA from unilateral moyamoya disease, in which stenotic change begins at the MCA. The knowledge of the anomalies of the MCA is important to perform a safe surgical or endovascular intervention.