We report this first case of synchronous cervical rhabdomyosarcoma and Sertoli-Leydig cell tumor (SLCT) of ovary with congenital absence of unilateral ovary and fallopian tube in a 16-year-old female patient. A review of the English-language literature identified only six cases of double malignancies with cervical rhabdomyosarcoma and SLCT but none of the case was associated with congenital absence of ovary and fallopian tube. When such multiple conditions involving one system occur in an individual, the possibility of single etiology arises, but beside some hypothesis, no definite link could be established in any of the cases reported.