Background: Swyer syndrome is a rare disorder of testis development caused by genetic mutations of genes responsible for the development of the testis. The chance of tumors in these patients is very high. The patients are young and usually tall women who present with primary amenorrhea and 46, XY karyotype. Given the absence of functional ovaries, secondary female characteristics are variable. This case report describes a young individual with Swyer syndrome, presenting with primary amenorrhea and diagnosed with a gonadoblastoma of the ovary. Early diagnosis and treatment of this syndrome are important to prevent and treat ovary tumors. Conclusion: Early diagnosis and treatment of Swyer syndrome are essential for preventing ovarian tumors. Furthermore, comprehensive management, including hormone replacement therapy and assisted reproductive technologies, is necessary to support normal sexual development and explore possibilities for pregnancy.