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Unlabelled: 46,XY gonadal dysgenesis was diagnosed in a 5.5-year-old phenotypically female patient who had physical and somatic stigmata of Turner syndrome such as webbed neck, low hairline, widely spaced nipples, cubitus valgus and coarctation of the aorta. Bilateral streak gonads were removed and an unsuspected gonadoblastoma was found in right gonad.Conclusion: The prepubertal development of gonadal neoplasm in patient with Xy gonadal dysgenesis indicated the necessity of gonadectomy at the time of diagnosis. [ABSTRACT FROM AUTHOR]