Choriocarcinoma is one of rare neoplasms of female reproductive organs. In the last decade only a few cases of this disease have been registered in Poland. The paper presents the current principles of diagnosis and treatment options for this rare disease based on the authors' clinical experience (description of the case) and literature review. This paper provides basic information on epidemiology, FIGO classification, and also discusses the chemotherapy regimens used in the medical treatment of choriocarcinoma. Surgical options were also considered. A thematic review of the most important papers published in international journals in recent years has also been made.