Background: For individuals with DSD, the decision of gender assignment at birth is a challenging one and the incidence of gender dysphoria in adulthood is much higher than that in the general population. Aim: Our goal is to provide strategies for gender assignment at birth for individuals with DSD and investigate the underlying reasons for gender transition in DSD patients. Methods: Conducting a retrospective clinical case analysis of DSD patients admitted to our department from 2000 to 2020, with a focus on analyzing the reasons for gender reassignment among these patients. Results: In 91 cases of 46, XY DSD, 71 were raised as females and 20 as males. Among them, gender transition occurred in 18 cases, with 17 female-tomale (FTM) transitions and one male-to-female (MTF) transition. In the case of 53 patients with 46, XX DSD, there were 39 who were raised as females and 14 as males. After diagnosis and treatment, a total of nine cases underwent gender transition, including seven FTM transitions and two MTF transitions. Clinical Implications: The choice of gender assignment at birth significantly impacts the occurrence of future gender dysphoria, emphasizing the importance of increased attention to individuals with DSD who experience gender dysphoria. Strengths and Limitations: This study provides a detailed analysis of the reasons for gender transition in individuals with DSD and offers some references for gender assignment at birth based on previous literature. However, the major limitation of this study is the lack of specific etiology for DSD patients who undergo gender transition. Conclusion: For children with abnormal external genitalia at birth, it is recommended that chromosome karyotype analysis and gene detection be performed immediately. A proper upbringing regarding gender can greatly reduce the occurrence of gender anxiety in patients and the resulting suffering.