BACKGROUND: NR5A1 plays essential roles in the development of various tissues, including the ventromedial hypothalamus, pituitary gonadotrope, adrenal cortex, spleen, testis, and ovary. Additionally, NR5A1-positive cells in these tissues exhibit developmental and functional heterogeneity. METHODS: This review summarizes recent knowledge on the relationships between physiological functions and gene cascades regulated by NR5A1 in each tissue. In addition, we also present several intriguing examples of disparities in MAIN FINDINGS: The adrenal cortex and testicular Leydig cells exhibit clear biphasic developmental processes, resulting in functionally distinct fetal and adult cell populations in which CONCLUSION: Some steroidogenic tissues demonstrate biphasic development, with fetal and adult cell populations playing distinct and crucial physiological roles.