Abstract
We report the case of a 14-year-old girl with xeroderma pigmentosum who was incidentally found to have a juvenile granulosa cell tumor of the ovary during routine imaging. The tumor was surgically removed, and no signs of hormonal activity or metastasis were observed. The patient had an uneventful recovery and required no additional therapy. This case underscores a rare but noteworthy association between a DNA repair disorder and early-onset gynecological tumors, highlighting the importance of regular gynecological surveillance in affected patients. Early detection may improve clinical outcomes and reduce the need for aggressive treatment.