Sickle cell disease (SCD) has been identified as one of the most prevalent genetic conditions. It alters the shape and function of red blood cells. This brief case report presents a case of a five-year-old male with sickle cell disease who complained of pain in the left mandibular region due to deep proximal caries. Before dental management, a complete fitness evaluation was performed with the help of a pediatrician, followed by informed consent. Dental management includes pulpectomy followed by stainless steel crown placement and Glass ionomer cement (GIC) restoration for superficial caries. Other oral manifestations were observed, including a smooth tongue and mucosal pallor. It was concluded that dentists and health professionals should be knowledgeable of the general and oral anomalies that can be present in individuals with sickle cell anemia in order to take preventive action and implement effective management.