Alport syndrome is an inherited kidney disease caused by mutations in the COL4A3, COL4A4, or COL4A5 genes. The disease is characterized by hematuria, proteinuria, hearing loss, and ocular lesions. In the last decade, our knowledge of the prevalence and diagnostic modalities of the disease has been updated with the popularization of genetic testing technology in medical practice. Despite the lack of etiologic treatment for Alport syndrome, early use of renin-angiotensin-aldosterone system blockers can dramatically improve the prognosis of patients. A variety of new therapeutic agents as well as therapeutic approaches are under investigation, which will bring new hope to patients. This article mainly reviews the latest progress in the clinical manifestations, diagnosis, and treatment of the disease, in order to achieve early diagnosis and treatment of the disease and optimize the clinical management for patients.