PURPOSE: Cutaneous lipomatous neurofibroma is a rare variant of neurofibroma. Histologically, it includes adipose tissue. As far as we aware, only 11 cases of this variant were reported which was predominantly on head, neck and trunk. Hence we present a case of left pretibial area with literature review. METHODS: The patient was a 17-year-old female who showed a non-tender, protruding 4x4x2cm sized mass on the left pretibial area for several months. We totally excised the mass including skin and the subcutaneous fat layer. RESULTS: Pathologic report showed cutaneous lipomatous neurofibroma which was well circumscribed and noncapsulated neoplasm present with focal fatty change. Adipose cells were entrapped in the whirls of spindle cells. There were no lipoblasts or atypical adipocytes. CONCLUSION: A cutaneous lipomatous neurofibroma on the lower extremity is very rare. In our patient, there were no trauma-related histopathologic changes. Therefore, focal fatty change can be a consequence of metaplasia from multipotential neural cells after migration.