Alveolar soft tissue sarcoma (ASPS) is a rare malignant soft tissue tumor with characteristic histological features, slow growth and a tendency to metastasize. The main molecular feature is the ASPSCR1-TFE3 gene fusion, which is crucial for diagnosing and understanding the pathogenesis of the disease. The diagnosis of ASPS involves histopathological and immunophenotypic examinations, which help distinguish this tumor from other sarcomas. Thanks to advances in molecular pathology and the use of multi-omic technologies, the accuracy of diagnosis is improving and the possibilities of personalized treatment are opening up. Clinical management of ASPS is challenging due to its tendency to metastasize, but new targeted therapies are improving patient prognosis. This approach enables better adaptation of treatment to the individual characteristics of the tumor and increases the chances of better results. The article emphasizes the importance of the integration of molecular knowledge into clinical practice for optimizing the diagnosis and treatment of ASPS.