For the first time in the English medical literature is presented an extremely rare transitional cell carcinoma of the round uterine ligament. Transitional cell carcinoma (TCC) of the ovary was first defined by Austin and Norris in 1987. Malignant primary tumors arising in the uterine broad ligament are extremely rare. To date, its pathogenesis is unclear.

In the discussion we focus on the pathomorphological and immunohistochemical analysis of transitional cell carcinomas in the genital tract and their differential diagnosis from transitional cell carcinomas of the urinary tract / ureter and bladder.

Due to the high malignant potential, the prognosis is unfavorable and requires optimal complex treatment, including radical surgery followed by systemic chemotherapy.