Cerebellar liponeurocytoma that has been recently identified as a distinct entity by the World Health Organization is characterized by areas of lipomatous differentiation and apparently by a favorable prognosis. In this paper, we described a case of 49-year-old female showing progressive clinical course inspite of a low labeling/mitotic index. We also review the relevant literature. Although, basically all reported cases share a similar histological pattern, i.e. focal accumulations of adiposities in an otherwise typical small cell tumor like central neurocytoma, some clinical properties such as (age, proliferative potential, therapy and survival) are not uniform. The exact biological behavior of this special variant tumor is established. Yet, this needs further confirmation on a large number of cases with longer follow-up periods.