Introduction: Soft tissue sarcoma accounts for 1% of malignant neoplasms detected in adult patients in Poland. Their primary localization in the urogenital system is rare. Aim of the paper: To present a case of rhabdomyosarcoma alveolare of the urogenital region in a 40-year-old female patient and conduct a literature review. Material and methods: The retrospective analysis involved medical records of the patient hospitalized in the Oncology Center in Krakow. The available literature on soft tissue sarcoma in the urogenital region was reviewed. Results: A 40-year-old patient presented at the Oncology Center with a lesion in the area of the external opening of the urethra. The histopathological examination of the specimen confirmed the presence of rhabdomyosarcoma alveolare. Since the surgical procedure was not radical, postoperative brachytherapy was conducted. Thirteen months later, a local relapse was diagnosed and removed together with a fragment of the urethra. In the edges of surgical cuts, neoplastic tissue was detected. Postoperative chemo- and teleradiotherapy were started. The literature review demonstrated few case reports of alveolar rhabdomyosarcoma of the urogenital region in adult women. Discussion: Rhabdomyosarcoma is a childhood sarcoma. The results of retrospective studies indicate poor prognosis in adult patients, and the alveolar type is characterized by an aggressive course. This case report confirms the tendency of the neoplasm to recur locally. Conclusions: Rhabdomyosarcoma alveolare of the urogenital region in adult women is a very rare and highly aggressive neoplasm that tends to recur. The rare occurrence of this disease indicates that it should be diagnosed and treated in specialized centers. The urogenital localization does not allow radical surgical treatment and limits postoperative radiotherapy. Individualized combined treatment is therefore necessary.