ABSTRACTA 19-year-old phenotypically normal female from Kakamega, Kenya presented with primary amenorrhea. Physical examination revealed Tanner stage 3 breast development, Tanner stage 4 pubic hair, and normal external genitalia. Hormonal profile tests indicated hypergonadotropic hypogonadism with normal 17-hydroxyprogesterone and testosterone levels. Ultrasound scan showed a normal uterus without ovarian pathology, while MRI revealed a hypoplastic uterus and absent ovaries. Karyotyping confirmed a 46, XY genotype, leading to a diagnosis of 46, XY Complete Gonadal Dysgenesis (Swyer syndrome). Swyer syndrome is a rare disorder of sex development characterized by unambiguous female genitalia, bilateral streak gonads, and elevated gonadotropin levels in individuals with a 46, XY karyotype. The condition results from abnormal gonadal development due to mutations in testis-determining factors, most commonly the SRY gene. Patients typically present with primary amenorrhea and lack of secondary sexual characteristics. Management includes hormone replacement therapy and gonadectomy due to the increased risk of gonadal tumors. The patient was educated about her condition, initiated on combined contraceptive pills, and counseled on exploratory laparoscopic gonadectomy. This case highlights the importance of a comprehensive diagnostic approach in patients with primary amenorrhea, including physical examination, hormonal evaluation, imaging, and karyotyping, to accurately diagnose and manage disorders of sex development.