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Introduction: Sickle cell disease is the most common monogenetic disorder worldwide. There have been reports of endocrine dysfunction and gonadal failure among affected individuals, especially in males. The findings on ovarian reserve and failure in women with sickle anaemia have been inconsistent. Aim and objective: The aim of this study was to determine and compare the ovarian reserve of Nigerian women with and without sickle cell anaemia attending a University Teaching Hospital. Study Design: This cross-sectional study was carried out at the Adult Sickle Cell Clinic and the Community Health Clinic of the Lagos University Teaching Hospital. Methodology: A total of 166 participants who met the selection criteria, were recruited for the study. The study population consisted of two groups of women matched for age: 83 women with HbSS and 83 women with HbAA. The haemoglobin phenotype of each participant was determined on alkaline electrophoresis (pH 8.4) before analysis. Serum Anti-Mullerian Hormone (AMH) was determined using Enzyme-linked immunosorbent assay (ELISA) method (Calbiotech Inc. USA, Catalog no AM448T). Results: The mean ± SD of serum AMH level in women with HbSS was 3.64 ± 0.65 ng/mL and was lower than that of women with HbAA 7.35 ±1.19 ng/mL (p < 0.001). Serum AMH negatively correlated with age in both study groups (HbAA and HbSS). Also, a significant negative correlation was found between serum AMH and BMI in women with HbAA. Conclusion: The study showed diminished ovarian reserve in women with HbSS when compared to age-matched women with HbAA. [ABSTRACT FROM AUTHOR]