We report anatomical and cosmetic results of feminizing genital reconstruction in patients with a high vagina due to disorders of sexual differentiation. Twelve patients with urogenital sinus anomalies graded as Prader IV underwent one-stage perineal clitoral vaginoplasty at a mean age of 1.6years. Seven patients had congenital adrenal hyperplasia, four partial androgen insensitivity and one mixed gonadal dysgenesis. Mobilized common sinus, opened dorsally without pubourethral ligament dissection, was used in combination with a perineal skin flap to construct the distal vagina. Clitoroplasty and labioplasty were done simultaneously. Mean follow up was 7.3years. In all cases the vaginal introitus was positioned in the vestibule region below the urethral meatus. One patient developed postoperative glans atrophy. Agreement between parental and physician satisfaction with postoperative cosmetic genital appearance was recorded in 11 girls. Vaginal stricture occurred in one patient, treated successfully with repeat vaginoplasty. One girl experienced urinary stress incontinence and became dry after bladder neck injection of a bulking agent. This procedure is successful in creating a feminine genital appearance in children having disorders of sexual differentiation with high vagina. Long-term follow up is needed to reassess the initial good anatomical and cosmetic results and evaluate sexual function after puberty.