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The Gorny Altai region in southern Siberia is one of the key areas in reconstructing the tectonic evolution of the western segment of the Central Asian Orogenic Belt (CAOB). This region features various orogenic elements of Late Neoproterozoic–Early Paleozoic age, such as an accretionary complex (AC), high-P/T metamorphic (HP) rocks, and ophiolite (OP), all formed by ancient subduction–accretion processes. This study investigated the detailed geology of the Upper Neoproterozoic to Lower Paleozoic rocks in a traverse between Gorno-Altaisk city and Lake Teletskoy in the northern part of the region, and in the Kurai to Chagan-Uzun area in the southern part. The tectonic units of the studied areas consist of (1) the Ediacaran (=Vendian)–Early Cambrian AC, (2) ca. 630 Ma HP complex, (3) the Ediacaran–Early Cambrian OP complex, (4) the Cryogenian–Cambrian island arc complex, and (5) the Middle Paleozoic fore-arc sedimentary rocks. The AC consists mostly of paleo-atoll limestone and underlying oceanic island basalt with minor amount of chert and serpentinite. The basaltic lavas show petrochemistry similar to modern oceanic plateau basalt. The 630 Ma HP complex records a maximum peak metamorphism at 660 °C and 2.0 GPa that corresponds to 60 km-deep burial in a subduction zone, and exhumation at ca. 570 Ma. The Cryogenian island arc complex includes boninitic rocks that suggest an incipient stage of arc development. The Upper Neoproterozoic–Lower Paleozoic complexes in the Gorno-Altaisk city to Lake Teletskoy and the Kurai to Chagan-Uzun areas are totally involved in a subhorizontal piled-nappe structure, and overprinted by Late Paleozoic strike-slip faulting. The HP complex occurs as a nappe tectonically sandwiched between the non- to weakly metamorphosed AC and the OP complex. These lithologic assemblages and geologic structure newly documented in the Gorny Altai region are essentially similar to those of the circum-Pacific (Miyashiro-type) orogenic belts, such as the Japan Islands in East Asia and the Cordillera in western North America. The Cryogenian boninite-bearing arc volcanism indicates that the initial stage of arc development occurred in a transient setting from a transform zone to an incipient subduction zone. The less abundant of terrigenous clastics from mature continental crust and thick deep-sea chert in the Ediacaran–Early Cambrian AC may suggest that the southern Gorny Altai region evolved in an intra-oceanic arc-trench setting like the modern Mariana arc, rather than along the continental arc of a major continental margin. Based on geological, petrochemical, and geochronological data, we synthesize the Late Neoproterozoic to Early Paleozoic tectonic history of the Gorny Altai region in the western CAOB.