Background: We present a case of giant hepatic hemangioma with Kasabach-Merritt syndrome. Material and methods: A thirty-seven-year-old female presented to us with a six-month history of recurrent abdominal pain and weight loss. A CT scan was suggestive of a huge right liver mass involving the entire right lobe, which was indicative of hemangioma. Her blood chemistry showed a platelet count of 68,000 and INR of 1.4, suggestive of Kasabach-Merritt syndrome. Results: In view of the size of the mass, symptoms and associated Kasabach-Merritt syndrome, the patient was offered surgical removal of the tumour. On exploration, the tumour covered the right lobe completely and so a right hepatectomy was performed. The hemangioma was 25 cm × 20 cm × 16 cm and weighed 1.8 kg. The postoperative course of the patient was uneventful. On day 3, the platelet count had improved to 98,000 and the INR had improved to 1.2. The patient was discharged on day five. Conclusions: A right hepatectomy for a giant hemangioma of the liver is a safe procedure and can be curative for associated Kasabach-Merritt syndrome.