The purpose of this study was to evaluate the association between sickle cell anemia (SCA) and pulp necrosis (PN).One hundred thirteen individuals with SCA (ie, the exposed group) from the Supervision of Hematology and Hemotherapy of Maranhão (HEMOMAR), Maranhão, Brazil, and 226 individuals without SCA, the sickle cell trait, or other diseases (ie, the nonexposed group) were enrolled in this study. All participants were over 16 years old and had at least 1 clinically intact permanent tooth. Patients with SCA and a history of lower alveolar nerve paresthesia and those who had suffered from vasoocclusive crises within the previous 6 months were excluded. PN of clinically intact permanent teeth without a history of orofacial trauma was diagnosed using the cold thermal test (CTT) and pulse oximetry adapted for dentistry (POD). Poisson regression was used to estimate the associations (P < .05).In an unadjusted analysis, the occurrence of PN in clinically intact permanent teeth was 8.33 times higher in the exposed group than in the nonexposed group (P < .001). This association remained significant after adjusting for a history of orofacial trauma and folic acid use for the CTT and POD evaluations.In conclusion, SCA is a potential risk factor for PN in clinically intact permanent tooth.