| Summary: | Precocious puberty refers to appearance of secondary sexual characteristics, increased growth rate and reproductive capacity before 8 years in girls, and 9 years in boys. The etiology can be diverse, either central activation of hypothalamus-pituitary gonadal axis or by endogenous production or exogenous administration of sex steroids. Peripheral precocious puberty is less common and Leydig-Sertoli cell tumors comprise 1–2% of the pediatric ovarian malignancies. (1) These tumors commonly present in the second or third decade of life but can present as early as 2-years of age.(2).We describe a case of precocious puberty caused by ovarian Sertoli-Leydig cancer in a young girl with no pre-existing medical issues. The 2-year-old patient was referred from Saint Martin to the Pediatric endocrinology for evaluation of pubic hair and breast development which started 3-month prior to presentation. On exam, the patient was noted to have abdominal prominence which prompted obtaining abdominal imaging which showed an ovarian tumor which was subsequently removed.
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