Spindle cell lipoma usually occurs as a solitary, subcutaneous, circumscribed lesion in the back, posterior neck or shoulders of older men. As a paratesticular tumor, spindle cell lipoma is extremely rare. Herein we report the second case of spindle cell lipoma of the spermatic cord in the published reports. The tumor comprised mature adipocytes and uniform spindle cells within thick ropey collagen bundles and mast cells. Immunohistochemical studies showed spindle cells positive for CD34, a diagnostic feature of this tumor.