| Objectives: To report a case of rare right renal agenesis and retroperitoneal schwannoma in right renal fossa, review the relative literatures, and analyze the clinical diagnosis and treatment.Methods: By analyzing the diagnosis, treatment, postoperative pathology and follow-up of the case retrospectively, and summarizing the papers, we investigate the characteristics of the disease for clinical reference.Results: The patient underwent completely tumor resection, and postoperative pathology confirmed that the tumor was retroperitoneal schwannoma. None evidence of recurrence or complication was found with a 6-month-follow-up after operation.Conclusions: Retroperitoneal schwannoma is difficult to be identified by imageological examination preoperatively. Pathology is the gold standard for its diagnosis. The ideal method for therapy is completely surgical excision. The prognosis is good, with a low rate of recurrence. Congenital renal agenesis is also hard to be discovered early. Long-term follow-up is need for that, and functional damage of the solitary kidney should be detected and treated timely. |