Women with sickle cell disease (SCD) are of particular concern regarding the significantly increased risk of pregnancy-related morbidity, mortality, and adverse outcomes. They have limited knowledge of pregnancy and childbirth risks, as well as of the benefits and risks of contraceptives. Thus, there is an urgent need for appropriate information about reproductive family planning to reduce unintended pregnancy. Any decision regarding the use of contraceptives has to be based on the efficacy and risk/benefit ratio of the method used. Both the World Health Organization (WHO) and the Centers for Disease Control (CDC) have developed, published, and updated evidence-based guidelines for medical providers for the use of contraceptives in patients with specific medical chronic conditions. This article provides an overview of the present knowledge on the use of contraceptives in women with SCD. We believe that the collaboration between health care professionals (hematologists, obstetricians, endocrinologists, and primary care providers) can play a major role in identifying the safer contraceptive method to abolish the risks of unintended pregnancy and preserve the health status of patients with SCD.