Methylmalonic acidemia (MMA) is a severe form of organic acidemia that can cause growth and developmental disorders, as well as multi-system and multi-organ damage. In China, over two decades have passed since Shanghai Xinhua Hospital pioneered the first neonatal tandem mass spectrometry screening techniques. Early screening and diagnosis of patients with MMA can now be achieved in the neonatal period, before clinical symptoms appear. Together with effective treatments, the survival rate and prognosis of MMA patients have greatly improved. However, as MMA is a metabolic disease that affects the whole body, patients are still at risk of neurological, renal, and other multi-organ complications. This article will elaborate on the clinical manifestations, pathogenesis, early detection and recognition, and treatment of renal complications in patients with MMA, with the aim of improving clinicians’ understanding of renal complications in MMA.