Abstract: Alveolar soft part sarcoma (ASPS) is a rare malignant tumor characterized by a lack of specific clinical manifestations, often presenting as painless, progressively enlarging masses. Its occurrence in the retroperitoneum is uncommon. Due to the large retroperitoneal space, the tumor typically does not cause significant symptoms in the early stages, leading to advanced disease or metastasis at presentation, and consequently, poor prognosis. This article reported the clinical diagnosis and treatment of a patient with retroperitoneal ASPS and elucidated the features of epidemiology, clinical manifestations, diagnostic modalities, and treatment approaches of this disease in conjunction with the literature.