Although the modern Indian and Javan rhinos have a single horn on their noses, the extinct one-horned rhino Elasmotherium was a source for the legendary unicorn, because the latter had a very long horn on its forehead and lived with the prehistoric human beings who drew its images on cave paintings. Elasmothere rhinos first appeared in South Asia in the Early Miocene, but the origin of Elasmotherium has been unclear. All other elasmotheres have a weak or strong nasal horn, whereas Elasmotherium seems to lose the nasal horn of its ancestors and to get a huge frontal horn apparently abruptly. Here we report the first discovered skull of Sinotherium lagrelii from the Late Miocene red clays in the Linxia Basin, northwestern China. This skull has an enormous nasofrontal horn boss shifted posteriorly and a smaller frontal horn boss, which are connected to each other, indicating an intermediate stage for the single frontal horn of Elasmotherium. Morphological and phylogenetic analyses confirm that Sinotherium is a transitional taxon between Elasmotherium and other elasmotheres, positioned near the root of the giant unicorn clade and originated in a subarid steppe. The posteriorly shifted nasal horn has a more substantial support and the arched structure of the nasofrontal area is an adaptation for a huge horn.