Introduction: The primary alveolar soft part sarcoma (ASPS) of bone is extremely rare, and the number of published reporting cases is limited. Case Report: We present a 30-year-old woman with primary ASPS of ilium. Computed tomography and magnetic resonance imaging revealed a destructive and moth-eaten appearance in the ilium with a soft tissue mass. 18F-fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) revealed high uptakes of FDG in the left ilium. ASPSCR1-TFE3 gene fusion was detected by reverse transcriptase polymerase chain reaction (RT-PCR). Wide resection was performed. One year after the surgery, a local recurrence developed. However, there has been no evidence of disease in the last eight years after the second surgery. Conclusion: 18F-fluorodeoxyglucose positron emission tomography-computed tomography and RT-PCR besides routine radiological and histopathological studies helped achieve the accurate diagnosis and provide appropriate treatment in the present case.