Background: Müllerian papilloma is a rare benign tumor of the female genital tract. It mostly affects girls less than five years old. After the treatment long-term follow-up is needed as there is a chance of recurrence, but even then the prognosis is excellent.
Case: A 19-year-old girl with Proteus syndrome presented with vaginal bleeding. The histological examination revealed Müllerian papilloma of the uterine cervix and large bilateral ovarian cystadenomas. The patient was treated with a radical operation, because there were signs of more aggressive behavior in the tumor. The patient is alive and free of disease five years after the operation.
Conclusion: The medical care of patients affected by rare disorders depends heavily on experiences gained from cases published in the medical literature. Since there is not much experience with tumors in Proteus syndrome we believe that this case can aid in shedding light on this subject.