Abstract: Homovanillic acid (HVA) and vanillylmandelic acid (VMA) are end metabolites of catecholamines. Measurement of HVA and VMA in urine not only serves as an indirect indicator of catecholamine secretion in the body but also holds significant importance in the early diagnosis and differential diagnosis of central nervous system diseases such as neuroblastoma and pheochromocytoma. Although various methods for detecting HVA and VMA are available, significant discrepancies exist in the results obtained from the same sample when tested within or across different institutions. Therefore, establishing reference methods and reference systems to ensure the accuracy and comparability of HVA and VMA measurement results, as well as traceability and standardization of testing, has become a new goal and challenge in the field of clinical detection. This article reviews the clinical significance, detection methods, and current status of standardization of HVA and VMA testing.