Chronic kidney disease occurs due to a progressive and irreversible decline in the total number of functioning nephrons, which causes a decline in the glomerular filtration rate. It is considered a major public health problem affecting the young population with a rapidly increasing global burden. Children suffering from chronic kidney disease have many oral manifestations resulting from the medical condition itself, the type of treatment administered, or both. Oral manifestations include a uremic odor, uremic stomatitis, glossitis, and gingival enlargement secondary to drug therapy. Other manifestations include enamel hypoplasia, increased dental calculus deposition, and xerostomia. Oral health care is important in the management of CKD. Negligence of oral health in children with CKD is due to poor dental awareness among the children and health care personnel which leads to poor quality of life for these children. This article reviews the oral and dental manifestations of chronic kidney disease to guide dentists in their management of these patients. Treatment planning should be done after consultation with nephrologists regarding specific precautions for each patient.