Presacral myelolipomas are rare, benign, asymptomatic tumors composed of mature adipose tissue and hematopoietic elements, but fewer than 50 cases have been reported in the literature. They are usually discovered incidentally during imaging studies and are often misdiagnosed as liposarcoma, which have a malignant nature, because the imaging findings of myelolipoma can be similar to those of liposarcoma. It is challenging to distinguish presacral myelolipomas from other presacral fat-containing tumors without performing a histological examination. We should consider the possibility of a malignant tumor, and imaging-guided biopsy carries a risk of tumor spread along the biopsy tract. Therefore, surgical management might sometimes be required; however, it is not necessary in all cases. We present an incidentally detected case of presacral myelolipoma that was difficult to differentiate from other malignant tumors in a 71-year-old male.