Eighteen girls with major uterovaginal malformations were admitted to the Pediatric Surgical Service over a 17-year period. The diagnosis was not suspected or delayed in more than one half of the patients. The encountered anomalies were divided into four groups: I, isolated uterovaginal malformations (UVM) (4); II, UVM with anorectal anomalies (8); III, UVM with cloacal (urogenital sinus) abnormalities (5); and IV, caudal twinning (1). Imperforate hymen, vaginal web, low vaginal obstruction, or disorders of gonadal or chromosomal development were excluded. Patients presented with an abdominal mass or distension (5), abdominal pain (4), "sciatic"-like pain (1), purulent vaginal discharge with perineal pain (1), amenorrhea (2), and a pelvic and prerectal mass (1). The introitus was reported as normal in 11 patients with vaginal atresia or agenesis by the primary physician. Diagnostic studies, in addition to clinical and endoscopic examination, included routine radiological workup, genitourinary contrast studies, pelvic and perineal sonography, computerized tomography (CT) scanning, and more recently, magnetic resonance imaging. In complicated UVM, especially vaginal duplications with unilateral atresia, the CT scan was the most helpful diagnostic tool. Laparotomy was necessary, not only for therapeutic, but diagnostic reasons; even so, complex anomalies, such as vaginal duplication with unilateral atresia and a septate uterus, could not be suspected. Treatment was directed toward the restoration of a functional uterovaginal tract and the frequently associated anorectal anomalies. A review indicated that contrary to our expectations, the major UVM occurred in children with a low imperforate anus rather than the high variety.