This is to report a case of a 13-year old female patient with a two-year history of an enlarging clitoris.

Pediatric endocrinology ruled out congenital adrenal hyperplasia. Examination of the vulva revealed a 2.5 x 4 x 3 cm hard, non-erythematous, non-tender bifid clitoral mass, with no inguinal lymphadenopathies on physical examination and pelvic ultrasonography. The patient underwent incision biopsy, rush frozen section, and partial clitoridectomy, and was subsequently readmitted for completion excision.

Frozen section revealed a spindle cell neoplasm. Final histopathology with immunohistochemistry revealed a granular cell tumor of the clitoris.

Granular cell tumors of the clitoris are predominantly benign, slow-growing and asymptomatic with a risk of malignancy in rapidly growing tumors >4cm. There is no recommended routine examination nor imaging studies for early detection. In all cases, complete excision of the mass is recommended. This is the first local case report of this disease to our knowledge.