We report on an incidental finding of a markedly tortuous basilar artery in a 6-year-old child. The child underwent a computed tomography scan for minor head trauma, and a basilar artery abnormality was discovered, i.e., a markedly tortuous basilar artery without any other congenital anomalies or syndromes. After an exhaustive workup including computed tomography, magnetic resonance imaging, magnetic resonance angiography, computed tomographic angiography, and genetic tests for associated genetic syndromes, no intervention was deemed necessary. The embryonic etiology and clinical implications are discussed.