Intramuscular lipoma (IML) is a relatively common variant of lipomas. The most important sites for IML are the large muscles of the extremities. Spindle cell lipoma (SCL) is a rare and distinct variant of lipoma. Most SCL arise in the neck, shoulders or back. It has also been described in unusual sites, such as the oral cavity, larynx, bronchus, breast, orbit and extremities. However, localization of a SCL in an IML has not been described yet. Thus, we present the first SCL located in an IML, which was localized underneath the fascia and embedded within the left sartorius muscle of a 55-year-old man. Microscopically, the SCL component of the tumor was sharply circumscribed by a fibrous capsule and clearly seperated from the IML in which it was localized. The collagen-forming spindle cells of the SCL showed neither atypia nor pleomorphism. These cells stained positive for CD34, while the mature fat tissue component of the SCL was positive for S-100 protein and negative for CD34. Spindle cells were negative for S-100 protein. Vimentin stained both components of the SCL, as well as the striated muscle fibers and mature fat tissue of the IML. In conclusion, careful morphological observation along with immunohistochemistry for CD34 and S-100 protein are essential to differentiate this rare tumor from lesions that enter the differential diagnosis.