Sex cord tumor with annular tubules is an extremely uncommon gonadal stromal neoplasm. It represents 0.05-0.6% of all ovarian tumors, according to series. An unusual case is presented in a 6-year-old girl, detected as a result of an isosexual peripheral precocious puberty. The highlight of this case is that no mass attached to the ovary was found, but only a gonadal asymmetry without radiological signs of malignancy. After confirming the presence of tumoral cells by intraoperative biopsy, unilateral salpingo-oophorectomy with ipsilateral para-aortic and pelvic lymphadenectomy was performed. Afterwards, the evolution of the patient was favorable.
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