Mesenchymal hamartoma is the second most frequent benign liver tumor in young children and usually occurs in the first two years of life. Currently, the gold standard for treatment is a complete surgical resection. We report a 17-month old patient with a giant mesenchymal hamartoma who, due to the impossibility of resection and the risk of malignant transformation, received liver transplantation from a living donor with a satisfactory outcome in the first year post-transplant. In this report, we show that liver transplantation is a viable therapeutic alternative for this pathology, in patients who have no possibility of receiving another type of treatment due to their clinical characteristics.