Background: Haemoglobinopathies are the most frequent single gene disorders worldwide, and particularly in the Eastern Mediterranean region, including Saudi Arabia. Information about the prevalence of sickle cell disease and thalassemia in the Southern region of Saudi including Al-Baha is not clear. The aim of this study was an attempt to determine the frequency of haemoglobinopathies among the individuals screened as part of the Saudi Premarital Screening Program at the Central Laboratory and Blood Bank Al-Baha, Saudi Arabia Methods: this was a retrospective cross-sectional study was conducted at the Central Laboratory and Blood Bank Al-Baha, Saudi Arabia as part of the National Premarital Screening Program. Reports of the years 1435 and 1436 Hijra (February 2014 to January 2016) were reviewed. Results: Totally 4,055 individuals were screened, the detected 278 cases with abnormal hemoglobin constitute (6.83%) of the screened samples during the period of the study ,with sickle cell trait (3.7%), followed by ß-thalassemia trait, sickle cell disease with (1.3 % ) and ( 0.99 %) respectively , while variant haemoglobin constitute only (0.86% ) Conclusion: We conclude that sickle cell anemia (SCA ) is the most common type of haemoglobinopathies among the population of Al-Baha region, and the screening program constitute the backbone of the preventive strategy for these haemoglobinopathies. Recommendation: Family screening of the SCA and SCT is necessary to identify sickle cell carriers that should be extend to all areas of Al-Baha region
