Abstract: Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor characterized by painless, non-functional, and slow-growing masses. Its rarity and deceptive growth pattern can make early diagnosis and timely management challenging in clinical practice. We report a case of ASPS in a 37-year-old male who presented with a five-year-old, initially undiagnosed swelling in his right axilla. Subsequently, the patient experienced intermittent dizziness, dysgraphia, and metamorphopsia. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass in the right axilla, multiple lung nodules, and a left parieto-occipital occupying lesion. Craniotomy confirmed metastatic ASPS in the intracranial region. Subsequent resection of the right axillary mass identified it as the primary tumor site. This article presents a literature review of ASPS, summarizing its CT, MRI, and clinical manifestations. We aim to enhance physician understanding of ASPS, providing insights for early diagnosis and treatment to improve long-term patient survival rates.