Currently, growing attention is being devoted to the conversion of biomass into value-added products, such as itaconic acid (IA), which is considered as the cleanest alternative to petroleum-based acrylic acid. IA is an unsaturated dicarboxylic acid that is used as a building block chemical for the production of several value-added products such as poly-itaconic acid. IA and its derivatives have a wide range of potential applications in textile, paint, pharmaceutical and chemical industries. Presently, industries are producing IA on the large scale by fermentation from glucose. However, due to the primary utility of glucose as a food, it cannot meet the global demand for IA production in an economical way. The main challenge, so far, has been the production technology, which does not support cost-effective and competitive production of IA. This review discusses the various bottlenecks faced during each step of IA production, along with possible remedies to deal with these problems. Furthermore, it reviews the recent progress in fermentative IA production and sheds light on different microorganisms used, potential substrates and fermentation conditions. The review also covers market potential for IA, which indicates that IA can be produced cost-effectively from sustainable substrates, and it has the potential to replace petrochemicals in the near future.