To investigate the characteristics, diagnosis, and treatment of ovotesticular disorder of sex development (OT-DSD).We retrospectively analyzed 2 cases of OT-DSD treated in our hospital. The patients were 19 and 15 years old, respectively, and both received systematic physical examination and examinations of the karyotype, sex hormone, adrenocorticotropic hormone (ACTH), color Doppler ultrasonography, urethrocystoscopy, and human chorionic gonadotropin (HCG) test. Under the laparoscope, we performed surgical gonad exploration, gonadectomy, and vulvar orthopedics. Intraoperative exploration and pathology confirmed true hermaphroditism in both cases, with sex selection as female. One underwent laparoscopic resection of the ovotestis, and the other removal of the testis with the ovarian tissue reserved.The patients were followed up for 12 months postoperatively, which found no abnormality in either the vulvas or the genital glands.Surgical exploration of the gonad is the only method for the diagnosis of OT-DSD and sex selection is the key to treatment. Laparoscopic surgical exploration of the gonad and vulvar orthopedics are the first treatment options.