Cystic fibrosis (CF) is the most common, life-threatening, autosomal-recessive disorder among Caucasians. Some of the mutations are very rare, and some represent individual sequence changes in the gene. In this case report, we present the dental treatment of CF in a 5 years old Saudi female. She has poor oral hygiene, poor dietary intake, generalized mild plaque induced gingivitis, multiple carious teeth, and a negative behavior. In addition, clinical and radiographic examinations indicated the presence of taurodontism and congenital absence of lingual frenum. We discuss the effect of the disease process on dental and oral health and management in the dental setting.