A 28-year-old woman with well-developed female secondary sexual characteristics, secondary amenorrhea, short stature and a few somatic anomalies of streak gonad syndrome who had bilateral ‘burnt out’ gonadoblastoma associated with 45, X karyotype is reported. Although the exact source of steroid production in the present case could not be defined, it seems probable that the tumor once secreted steroid hormones which were responsible for the development of the female secondary sexual characteristics. At the time of diagnosis there was no evidence of hormonal activity of the tumor. Cases of gonadoblastoma without Y chromosome are reviewed. The diagnostic difficulties encountered in patients with gonadoblastoma not associated with Y chromosome are discussed.