Congenital porto‐systemic shunts in sixteen dogs and three cats are described. A major symptom was hepato‐encephalopathy. In most cases diagnosis was based on elevated venous ammonia levels, abnormal ammonia tolerance and results of operative mesenteric portography. Clinical signs, laboratory findings, results of portography, and pathologic findings in the liver and the central nervous system are described. A diagnostic schedule is proposed to aid in recognition of this syndrome.