BACKGROUND: Granulosa cell tumor of the ovary is very rare in childhood; its most common clinical manifestation is isosexual precocious puberty. Clinical presentation as acute abdomen due to pain and ovarian torsion is rare, but a granulosa cell tumor must be suspected in a patient with this acute presentation and signs of early puberty. Adult-type granulosa cell tumor is an even rarer occurrence in children.

CASE REPORT: We report a case of torsion of adult-type granulosa cell tumor of the ovary in a 5-year-old patient with acute abdominal pain and ovarian torsion and highlight the importance of histological diagnosis of this tumor for the therapeutic plan and progression of these patients.

CONCLUSIONS: Precocious puberty, pain, abdominal distension, and an ultrasonography with suspicion of ovarian torsion are warning signs that may indicate the presence of a gonadal stromal tumor in pediatric patients seen at an emergency unit. These patients require long-term follow-up by a pediatrician and gynecologist because of the potential for late recurrence.

Keywords: Abdomen, Acute, Child, Granulosa Cell Tumor, Ovarian Neoplasms, Puberty, Precocious, Torsion Abnormality, Child, Preschool, Salpingo-oophorectomy