After winning prestigious international maths prizes as a school student, he began a science degree at the University of Western Australia when he was just 13 years old.
Three years later he became one of the university's youngest graduates after completing his degree, with first class honours, 12 months earlier than expected.
His mother Svetha Venkatesh, a professor of computer science at Deakin University, says her son was always a "normal, happy child growing up in a normal family" who was good at maths but also loved sport, music and reading.
"I am thrilled he has got this award, it's the pinnacle of what he can achieve in this field," she told ABC radio.
"And I'm actually very proud of the person he is."
Prof Venkatesh won a Hackett Scholarship from UWA to undertake a PhD at Princeton in 2002.
He is currently working as a professor of mathematics at Stanford, and plans to return to Princeton University this year.
The Fields Medal is only awarded once every four years to between two and four researchers under the age of 40 in recognition of their mathematical achievement.
Australian Mathematical Sciences Institute director Professor Geoff Prince said Prof Venkatesh was a worthy recipient of the award given his contribution to improving mathematicians' understanding of analytic number theory, algebraic number theory, and representation theory.
"Akshay is an exciting and innovative leader in his field whose work will continue to have wide-ranging implications for mathematics," Prof Prince said.
The only other Australian to have been awarded the Fields Medal was Professor Terrence Tao in 2006.