RESUMO
BACKGROUND: Ovarian steroid cell tumor, not otherwise specified (NOS), is a rare type of sex cord stromal tumor, which often presents with androgenic symptoms and has a high frequency of malignancy. CASE: This is a case of a 14-year-old Native American girl who presented with acne, amenorrhea, and virilization and was found to have a 2.9-cm solid ovarian mass. Initial pathology revealed steroid-appearing cells with round nuclei, clear/vacuolated cytoplasm, and a low mitotic index. Final diagnosis was ovarian steroid cell tumor, NOS Stage IA. A laparoscopic left salpingo-oophorectomy was subsequently performed. No tumor recurrence was noted 2 years after her initial diagnosis. SUMMARY AND CONCLUSION: Long-term data on these tumors are limited; however, malignancy, recurrence, and death have been reported. This suggests that close follow-up is essential for appropriate management.