One hundred and fifteen patients underwent orthotopic liver transplantation (OLT) for primary liver malignancy. Overall survivals of these patients were significantly lower than those of patients with non-malignant diseases (5-year survival rates 37% and 65%, respectively). Hepatocellular carcinoma (HCC) was the most common malignancy among our patients (n = 80). Fibrolamellar HCC (n = 9) was associated with better survival than non-fibrolamellar HCC (N = 71) among the lesions greater than or equal to 5 cm in diameter. More frequent recurrence was noted in patients with large tumors (greater than or equal to 5 cm), multiple tumors, and gross vascular involvement. A significant lower survival rate was observed in patients with bile duct cancer (n = 19) than in those with HCC or epithelioid hemangioendothelioma (n = 8). Careful patient selection and effective adjuvant anti-cancer therapy are needed to improve the results of OLT for primary liver malignancy.