He was the eldest surviving son of Emperor Michael Asen III "Shishman" and his first wife Ana ("Ana-Neda"), a daughter of King Stefan Uroš II Milutin of Serbia.
[1][2] According to an early interpretation of a rare coin type, it was believed that after his father's accession to the throne in 1323, Ivan Stefan was briefly associated as co-emperor; when Michael Asen III divorced Ana to marry Theodora Palaiologina, the daughter of Byzantine emperor Michael IX Palaiologos, in 1324, Ivan Stephen would have lost this position, as he was exiled along with his mother and brothers.
[7] The Serbian king met them in the Mraka area and accepted the proposals, making Ivan Stephen, by now his father's oldest surviving son, emperor of Bulgaria.
[8] Ana and her sons were informed of the agreement and escorted to the Bulgarian capital Tarnovo, where they were installed in August or September 1330; Ivan Stephen's uncle Belaur remained a prominent and supportive figure, apparently governing Vidin.
[22] Another brother of Ivan Stephen, Šišman, had fled to the Mongol Golden Horde in 1331 and reappeared in 1341 at Constantinople, where he was propped up as a potential claimant to the Bulgarian throne.
Later Ragusan chroniclers, including Giacomo Luccari, Mauro Orbini, and Giunio Resti, conflated local traditions about him with information about his brothers Šišman (whom they knew from Byzantine sources) and Lodovico, as well as an impostor, Nicholas Zap(p)in(n)a, producing a set of confused and implausible narratives.
For example, the renowned genealogical compendium of Detlev Schwennicke has Ivan Stephen settle in southern Italy, marry the illegitimate daughter of Philip I, Prince of Taranto, join the future Byzantine emperor John VI Kantakouzenos during his escape from Constantinople in 1342, fall a captive at Siena in 1363, and die at Naples in about 1373.