BACKGROUND Mesenchymal hamartoma of the liver is a rare benign liver tumor in children, usually arising from the right liver lobe and represents about 5 to 6% of all primary hepatic tumors. Complete surgical resection of the tumor is curative. CLINICAL CASE A 30 months old male presented with epigastrium abdominal pain and a palpable mass over a period of two days with no other symptom. The mass was excised completely. Postoperatively the patient recovered with an uneventful course and was discharge 13 days following surgery. All microscopic findings were consistent with the diagnosis of mesenchymal hamartoma of the liver. CONCLUSIONS Approximately 75% of mesenchymal hamartoma of the liver occur in the right lobe of the liver. Several diagnostic considerations should be elucidated to differentiate these type of tumors in the left lobe from other benign liver tumors. Sometimes a multidisciplinary approach is necessary to complete a successful complete surgical excision. Our case exemplifies a rare entity in a rare location, an adequate treatment in a third level reference hospital setting.