We report a rare case of montelukast sodium-induced hematuria in a 58-year-old female patient with allergic rhinitis. Renal function returned to normal after drug withdrawal. We reviewed 19 case reports of adverse reactions associated with montelukast sodium in mainland China and found that (1) 37% of patients were pediatric patients, (2) psychiatric disorders, rashes and uropoietic organs symptom were common, (3) uropoietic organ symptoms reported in mainland China were very rare abroad. The clinician in China should be vigilant about the adverse reaction of montelukast sodium and further studies are needed to explore the pathogenesis of montelukast-induced uropoietic organ symptoms.