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The goals of this study were to investigate the incidence and characteristics of hematuria in patients with microtia, and to clarify that more attention should be paid to renal dysfunction in patients with microtia. We conducted a retrospective cohort study of a total 9447 children diagnosed with microtia (selected as study group, 7037 children) or pigmented nevus (selected as control group, 2410 children) at the Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from January 2009 to June 2021. All of the routine urinalysis report of these children were reviewed to assess the incidence and characteristics of hematuria in each group. No statistically significant differences were observed when analyzing the overall incidence of hematuria between the study and control groups (P > 0.05). However, after grouping by sex, the incidence of hematuria in female children with microtia was significantly higher than that in femalecontrol group and no similar results were observed in the male patients. In addition, after further grouping by age in case group, the incidence of hematuria in girls of all ages with microtia was significantly higher than that in males with microtia (age 0–10:males: Girls = 1.89%:4.14%; age 0–5: males: Girls = 1.22%:3.73%; age 6–10: males:Girls = 1.97%:4.14%,P < 0.05), while no similar results were obtained in the control group.(age 0–10:males: Girls = 1.39%:2.22%; age 0–5: males: Girls = 1.07%:1.95%; age 6–10: males: Girls = 3.38%:3.17%, P > 0.05). Higher incidence of hematuria was observed in female children with microtia. [ABSTRACT FROM AUTHOR]