A case of Turner's syndrome clinically and cytogenetically diagnosed in a 15-year old girl is reported. At age 4 the girl was hospitalized for renal malformations (doubling of the urethra and its ectopic opening). Laparatomy revealed a small tumoral formation which was removed. The diagnosis established anatomo-histologically was of sympathoblastoma. Since the tumor is genetically conditioned by an autosomal mutation, association of the gonosomial anomaly and sympathoblastoma is supposed to be fortuitious though it is not improbable that the tumor be the result of an embryonar disorder favourized by the chromosomal anomaly.
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