Objectives Phyllodes tumor of the vulva is a rare condition, potentially arising from extra-mammary glands or mammary-like tissue, with less than 20 cases described in the literature. Patients from 17yo to 69yo usually present a painless tumor, with no other symptoms. Surgical complete removal is the current mainstain therapy.

Objetive To describe the diagnostic and therapeutic management of a Phyllodes tumor of the vulva occuring in a 15 year old female.

Methods The patient presented a pedunculated vulvar mass on the left labia majora, with 8 cm in the largest diameter. CT scans suggested a localized non invasive tumor. A margin free local resection was performed. Pathology report was conclusive for a Phyllodes tumor, with positive estrogen and progesterone receptors, cytokeratin AE1/AE3, and alpha smooth-muscle actin. Ki-67 index was 1%. Two years later, she recurred in the contralateral labia majora, with two pedunculated lesions (2 cm and 1.5 cm). A surgical resection was proposed, with perioperative frozen-section analysis of the margins.

Results Final pathology reports revealed a new diagnosis of Phyllodes tumor of the vulva, with free margins. There was a partial surgical site dehiscence, successfully managed conservatively. The patient is free of disease 8 months after last surgical resection.

Conclusions In this case report, an extremelly rare condition was diagnosed in a very young patient, with a contralateral recurrence, unusual for this type of tumor. Management was succesfull, with free margins local resections.