One in 300 to 4500 infants is born with abnormalities of the external genitalia and Mixed Gonadal Dysgenesis is the second most common cause. This is a case of a 12-year old child, raised as male and diagnosed to have ambiguos genitalia at birth. Analysis showed a mosaic karyotype, one cell line with 45 chromosomes including a Monosomy X and a second cell line showed a normal 46, XY karyotype. A thorough evaluation by a multidisciplinary team composed of a reproductive endocrinologist, pediatric gynecologist, pediatric urologist, pediatric endocrinologist, geneticist and child psychiatrist was made. The patient underwent pre-operative diagnostic cystourethroscopy and genitoscopy, total laparoscopic hysterectomy with bilateral gonadectomy and first stage repair of hypospadia. Laparoscopic management was the best approach for this patient because it provided minimally invasive surgery for children and enabled all necessary procedures, including evaluation, biopsy, and gonadectomy, for diagnosis d treatment.