This report presents a case of an eight-year-old girl affected by a progeroid syndrome of unclear genetic origins. The patient’s dental history included oligodontia, premature deciduous exfoliation and roots abnormalities. She was treated with comprehensive oral rehabilitation using dentures. Oral health instructions were given during the whole treatment and follow-up period. The goal of improving the masticatory function and the esthetic was achieved, allowing the patient to increase her social abilities and self-confidence.