Drash syndrome is characterized by nephropathy, Wilms tumor and male pseudohermaphroditism. We present a child with an incomplete form of this syndrome who underwent two operations. A full-term, 3070 g female was born after a normal transvaginal delivery. At the age of 1 year and 6 months, in order to confirm the diagnosis of the infantile nephrosis, a renal biopsy was performed with halothane anesthesia. Histological findings showed diffuse mesangium sclerosis. At the age of 3 years and 8 months, a chromosomal analysis revealed XY gonadal dysgenesis. Therefore, a diagnosis was made as the incomplete Drash syndrome. At the age of 5 years and 7 months, a gonadectomy and a CAPD catheter insertion for renal failure were performed under sevoflurane anesthesia. The main problems encountered by anesthetists were severe renal dysfunction, use of steroids, anticoagulants and immunodepressants, choice of anesthetic agent, and mental health care. This patient has been followed for the potential development of Wilms tumor.