As its name implies, hepatocutaneous syndrome (HCS) is a rare, progressive canine disease that manifests as distinct cutaneous and hepatic lesions. While many mechanisms explaining the disorder have been proposed, the pathophysiology is still not fully understood. Recently, research has proven that a marked hypoaminoacidemia is a characteristic of the syndrome. Moreover, clinical signs are not caused by primary hepatic dysfunction but rather alterations in hepatic amino acid metabolism. Such discoveries are crucial for the implementation of successful treatment approaches for this disease and may explain why HCS has traditionally had a poor prognosis. Patients with this syndrome should not be managed as a dog with hepatic insufficiency. Rather, intravenous amino acid therapy has been proven to be the most effective treatment. This report presents a case study of a 9.5 year old dog with HCS and attempts to provide a comprehensive overview of the disease.