Mesenchymal hamartoma (MH) is not an uncommon tumor of the liver in the age group of 2-10 years. It is the second most common benign liver tumor in children. Previously considered a developmental anomaly, newer insights into other theories of origin including toxic-metabolic, ischemic, and a true neoplastic process are in progress. Previous understanding of a purely benign nature of the tumor is being overridden by a real malignant transformation. Complete excision of the tumor with clear margins is recommended to achieve a long term cure. A thorough understanding of the natural history of these tumors and skillful surgical treatment are indispensable elements of care.