KBG syndrome is a rare, multiple congenital anomaly/mental retardation (MCA/MR) syndrome characterized by neurological disturbance, short stature, a distinct craniofacial appearance, and skeletal anomalies. It is likely to be autosomal dominant in nature with a wide range of expressivity in its clinical features. Dentally, macrodontia, particularly of the maxillary permanent central incisors is a common finding. The aim of this article was to review the familiar clinical presentations of this syndrome and to highlight previously unreported findings of generalized macrodontia and shovel-shaped incisors. Dental and clinical findings of 2 affected brothers with a 3-year follow-up of their dental progress following orthodontic treatment are outlined. Additionally, dental and clinical findings of an affected mother and her daughter, and another sporadic case are also presented.