We describe the case of a 3-year child in which pancreatic and parotid gland involvement preceded the development of the classical clinical phenotype of a typical Kawasaki disease (KD). The child was referred to the Emergency Department with a story of 3 days of continuous fever associated with abdominal pain and bilaterally swelling in the parotid regions; laboratory evaluation identified markedly increased levels of total amylase, pancreatic amylase, lipase, and transaminase, and diagnosis of pancreatitis was posed. After 9 days of fever and persistence of the clinical features, the classical signs of KD appeared, and the child was treated with intravenous immunoglobulins (IVIG), showing a dramatic response with complete resolution of the clinical picture. In this work, we reviewed the literature about gastrointestinal (GI) symptoms in KD, focusing on pancreatic and hepatic involvement. This analysis highlighted that, in case of fever associated with pancreatic inflammation, KD must be considered in the spectrum of differential diagnosis, and that GI involvement in KD is frequently associated with an incomplete response to IVIG treatment.