Pregnancy in sickle cell disease is associated with increased risk of maternal and fetal morbidity and mortality. Sickle cell disease is very common in tribal populations. The objective of this study was to review the maternal and perinatal outcome in patients with sickle cell disease of tribal populations. This is a retrospective study. The data extracted from the patients' case files included age, gravidity, family history, complications during pregnancy or at time of delivery or postpartum period, mode of delivery, and fetal outcome. There were 25 deliveries to women with sickle cell disease and 54 with sickle cell trait. Preeclampsia and disseminated intravascular coagulation were common problems associated with sickle cell disease as compared to the sickle cell trait and normal groups. No maternal mortality occurred during the period under study. However, a total of five intrauterine fetal deaths and one early neonatal death did occur. The present study confirms the previous reports, the increased risk of fetal death in women with sickle cell disease, however, in contrast to previous studies, no maternal mortality was found.