Portosystemic shunts (PSS) present in various ways, depending on their anatomic configuration and etiology. The most common class of PSS is congenital shunts, which can be divided into extra- and intra- hepatic. Extrahepatic shunts are more common in small breed dogs, whereas intrahepatic PSSs, like the one described in this case report, are more commonly seen in large breed dogs. They are typically the result of either failure of closure of an embryologic vessel - the ductus venosus - or they form during development of the portal vasculature and hepatic sinusoids. The three main body systems that the PSS effects are the nervous, gastrointestinal, and urinary tract with neurologic changes being most common. Medical management and surgical options are available for treatment. This report will discuss the classifications, clinical signs, diagnostics, and treatment options for portosystemic shunts.