Three-dimensional (3D), two-dimensional (2D) ultrasound and saline infusion sonography (SIS) are beneficial tools in diagnosing congenital uterine anomalies. This article illustrates six various case scenarios based on 3D ultrasound images in order to review the concepts of ultrasound diagnosis of congenital uterine anomalies and apply it to the presented case studies. These cases scenarios educate the reader on arcuate uterus, septate uterus, bicornuate uterus, didelphic uterus, uterus duplex and unicornuate uterus. This article also presents the sequence of embryologic events leading to the congenital uterine anomalies. The role of 3D ultrasound in diagnosis and treatment of congenital uterine anomalies is presented, along with its advantages over 2D ultrasound, SIS, X-ray hysterosalpingography, magnetic resonance imaging, hysteroscopy and laparoscopy. The effects of hysteroscopic metroplasty on fertility are also illustrated and discussed. After this case-based discussion is completed, the readers will be able to make a differential diagnosis of the different types of congenital uterine anomalies.