The authors describe the case of a young female patient with primary amenorrhea and normal secondary sexual characteristics. The diagnostic investigation demonstrated the occurrence of Mayer-Rokitansky-Kuster-Hauser's syndrome. Keywords: Paramesonephric (Müllerian) ducts; Abnormalities.Os autores descrevem o caso de uma paciente jovem com amenorreia primária e caracteres sexuais secundários normais. A investigação diagnóstica constatou a ocorrência da síndrome de Mayer-Rokitansky-Kuster-Hauser. Unitermos: Ductos paramesonéfricos; Anormalidades.
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