A personal case of persistent hypoglossal artery is presented and the literature on the subject from 1967 to the present reviewed. The 16 cases collected, following on the 26 already reported in the well-known monograph of T.A. Lie on congenital anomalies of the carotid arteries, complete the statistical picture of this malformation. After discussing the embryonic origin and describing the radiological anatomy of the anomaly, the authors briefly present their case and discuss, in the light of the cases collected, the possible role of persistent hypoglossal artery in the pathogenesis of vascular diseases of the head.