Abstract
OBJECTIVE: Granulosa cell tumor (GCT) is a rare entity of ovarian malignancies. Juvenile GCT is considered a malignant tumor with an indolent course and tendency toward late recurrence. However, the association of this tumor and multiple enchondromas has been reported. CASE PRESENTATION: A 17-year-old female with abnormal uterine bleeding was referred to our center. Ultrasonographic evaluation revealed a mass with origin in right ovary. Patient was worked up to undergo salpingo-oophorectomy, she felt a dull pain in her left lower limb. X-ray imaging was indicative for Ollier's disease at the distal part of femur and proximal part of tibia. Postoperative pathological review was compatible with juvenile granulosa tumor of the right ovary. CONCLUSION: This case was the first of its kind that ovarian tumor was contralateral to the side involved by enchondromatosis.