Campomelic syndrome is a rare autosomal recessive disease. It is characterized by short stature with angulation and bowing of the lower limbs, hypoplasia of the facial bones, and various other skeletal anomalies. The facies are unusually flat with micrognathia, frequent cleft palate, hypertelorism, and micro-ophthalmia. Most infants with this syndrome die from severe respiratory distress within the first hours or days of life. Those who survive infancy are mentally retarded. This case report concerns the dental treatment of a 13-year-old female with campomelic syndrome.