Kenny-Caffey Syndrome (KCS) is an extremely rare osteosclerotic bone dysplasia associated with hypocalcemia and ocular abnormalities. Although the condition is well reported in the medical literature, dental manifestations have not been discussed in great detail. The purpose of this report is to present specific oral features and prosthetic management in a KCS patient. Overlay dentures were utilized in the management of low vertical dimension of occlusion, congenital absence of several permanent teeth, and problems associated with function and esthetics. Results of the 4-year follow-up overlay denture therapy are presented.