The sideroblastic anemias comprise a heterogeneous group of acquired and congenital disorders which that have in common: (1) anemia; (2) the presence of ring sideroblasts in the bone marrow; and (3) impaired heme biosynthesis. Elevation in iron levels is secondary to a deficiency in mitochondrial physiology. Patients exhibit mild to moderate hemolysis due to peripheral red blood cell destruction, and often require regular blood transfusions. The purpose of this case report was to describe the dental management of a child with congenital sideroblastic anemia--a rare hematologic disorder--and to emphasize how important it is for pediatric dentists to be cognizant of a patient's hematologic profile before attempting to perform any dental procedure. The timing of the patient's transfusions regarding the scheduling of dental care is crucial for successful management and anticipation of possible complications. Preventive dentistry should be a hallmark of dental management for these children.