The present study investigated the benefits of intraoperative ultrasonographic guidance during the surgical repair of congenital cystic spinal dysraphic lesions. Twenty-one children with cystic spinal dysraphism who underwent surgical repair were examined by real-time ultrasonography during the surgical intervention. Five children had meningoceles, six had myelomeningoceles, four had open neural plaques, three had lipomyelomeningoceles, and three had diastematomyelia. Visualization of the cystic compartments, identification of the neural structures, and identification and localization of the associated lesions were all reliably achieved in all cases. Intraoperative ultrasonographic guidance could determine the type of lesion and the associated lipomas, ectopic tissues, dermoid and epidermoid cysts, and doubling of the spinal cord, and locate diastematomyelic spurs, bands, and adhesions. Components filled with cerebrospinal fluid appeared as anechoic areas, and lipomas as hyperechoic. Intraoperative ultrasonographic guidance allowed the surgeon to correlate the complex anatomy identified on preoperative computed tomography and magnetic resonance imaging to the surgical site during the operation. Better orientation to the defect allows appropriate repair of the lesion with optimal preservation of neural tissues.