A 10-year-old Maltese neutered female was presented for lip dermatitis and mild generalised pruritus. On examination, there were multiple lesions affecting the peri-oral and peri-nasal mucocutaneous junctions as well as distal limbs. Serum chemistry and a bile acids test were indicative of hepatocellular injury and hepatic dysfunction. Plasma amino acids were markedly depleted. Ultrasonography of the liver revealed a honeycombed appearance. A CT scan was performed to rule out a porto-vascular abnormality, hepatic or pancreatic neoplasia. Abnormalities in hepatic architecture are described in this confirmed case of hepatocutaneous syndrome (HCS) for the first time on CT. This is the first reported case of HCS in Hong Kong. The dog was treated with a high-quality protein diet and amino acids transfusions. Eleven months after presentation the dog was still alive with good control of clinical signs.