In order to clarify the true nature of rare lipomatous differentiation in MLS, we studied eight cases of MLSLC, by using immunohistochemistry (MDM2, CDK4, PPARγ, C/EBPα, adipophilin, perilipin, and Ki-67), chromosome analysis, fluorescence in situ hybridization (FISH), and reverse transcription polymerase chain reaction (RT-PCR). The gene discussed is CDK4; the disease is McLeod neuroacanthocytosis syndrome.