Juvenile xanthogranuloma is a histiocytic tumor that usually appears at birth or during the first year of life. Although most patients have solitary lesions, some have multiple cutaneous lesions, associated visceral lesions, or both. Although juvenile xanthogranulomas are usually confined to the skin; visceral involvement does occur in a small subset of patients and can be life threatening. We describe a newborn girl initially described as a “blueberry muffin baby” with multiple juvenile xanthogranulomas of the skin and liver that were complicated by progressive cholestasis and portal hypertension necessitating a liver transplantation.