Hepatic myelolipoma (ML) is a very uncommon finding. In a recent review of literature, only six cases of ML are reported. It is composed of adipose tissue associated to bone marrow elements. The CT, the echography and the aspecific symptomatology, if present, do not allow a sure diagnosis, which must be confirmed by histological examination of multiple tumor tissue samples. We report here a case of IM observed in a 53-year-old woman. The neoplasia was incidentally discovered after an echography performed after the onset of an aspecific painful symptomatology. Histologically, we have observed in this very rare tumor some features differing from those previously reported in literature, as the presence of an incomplete capsule and some "adenomatous" aspects of the surrounding hepatic tissue. The resulting differential diagnoses are reported and discussed. In particular, the presence of lipoblasts in the adipose tissue component requires great attention in order to avoid an erroneous diagnosis of sarcoma.