Spindle cell lipoma (SCL) is a rare lipoma variant that account for approximately 1.5% of all adipocyte-origin tumors; SCL usually occurs on the posterior neck or shoulder. The histological characteristics of SCL include mature, univacuolar fat cells and fibroblast-like spindle cells in a matrix of collagen and mucoid material. It is important to note that spindle cell lipoma can be mistaken both clinically and histologically for liposarcoma. We report here on a rare case of SCL in a 48-year-old male, and the patient presented with a large right neck mass that involved the lateral neck space and larynx.