Kawasaki Disease (KD) or atypical KD (AKD) rarely presents with intestinal obstruction or pseudo-obstruction. Others have reported gastrointestinal symptoms appearing with and up to 4 weeks after the occurrence of major clinical symptoms of KD. However, we presented a 1-year-old boy with prolonged fever who was found to have pyuria and liver dysfunction on the fourth day of fever. He developed a picture of intestinal pseudo-obstruction including bilious vomiting and abdominal distention on his fifth day of fever, four days before he developed the fissured lips. Because of the emergence of four major clinical criteria of KD, coronary artery dilatation, and aseptic meningitis, AKD was initially diagnosed and prompted the use of intravenous immunoglobulin on the tenth day of fever. Hydrops of the gallbladder, leukocytosis, increased ESR, and thrombocytosis were noted during hospitalization. These atypical features rarely develop all together in one patient with KD or AKD. Nevertheless, our patient had early intestinal pseudo-obstruction with almost all of the above unusual systemic manifestations and the late occurrence of clinical features of KD. We demonstrate that intestinal pseudo-obstruction in KD may develop earlier than other major clinical features and may improve under the conservative treatment. Surgery should be reserved for those who have complete intestinal obstruction presenting with significant peritoneal signs.