Abstract: Objective To summarize the characteristics of patients with congenital solitary kidney(CSK). Methods Medical data and post-operative follow up results of patients that was diagnosed with congenital solitary kidney in Peking Union Medical College Hospital from 2012 to 2020 were collected. Results A total of 233 patients with congenital solitary kidney (58 males and 175 females) were included in this study.The most common deformity was genital tract deformity followed by congenital scoliosis.15% of CSK patients had other renal disease.Urological surgery was performed in 5 patients,and no postoperative complications occurred during follow-up. Only 1 patient presented recurrence and metastasis 1 year after partial nephrectomy and underwent secondary surgery. Conclusions When congenital solitary renal malformation is screened out, it is necessary to carry out systemic evaluation.As for surgical treatment,it should be carefully executed,and long-term follow-up should be conducted to monitor renal function.

Key words: congenital solitary kidney, genital deformity, urinary disease, surgery