The article presents the case of rare combination of primary-multiple gonadal tumors: malignant disgerminoma and tumor that originating from Sertoli-Leydig cells with the syndrome of complete insensitivity to androgens. According to literature this kind of primary-multiple gonadal tumors are only 0,5%. Literature does not describe cases of such combination of malignant tumors and the syndrome of complete insensitivity to androgens. Proposed clinical observation demonstrates the need for

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... n in the risk groups for the development of gonadal tumors in girls with primary amenorrhea and dysgenesis of the gonads as well as the appropriateness and importance of medical genetic counseling in timely diagnosis and prevention of non-epithelial gonadal tumors.