Fibropolycystic liver disorders (FLD) arise from abnormal development of the ductal plate and are classified according to the size of the affected hepatobiliary duct. Congenital hepatic fibrosis (CHF) has small duct involvement characterized by a variable degree of periportal fibrosis and hyperplasia without affecting the liver's architecture. Caroli's disease (CD) is a rare autosomal recessive disorder with a prevalence of one case per 1,000,000 people and is characterized by cystic dilation of large intrahepatic ducts. When the disease presents with congenital hepatic fibrosis, it is referred to as Caroli's syndrome (CS). Patients are usually diagnosed around the age of 20 with episodes of cholangitis, portal hypertension or hepatomegaly. We present the case of a two-year-old male with a previous history of autosomal recessive polycystic kidney disease (ARPKD) who presented to the emergency room with variceal bleeding secondary to portal hypertension. The physical examination showed an acutely ill-looking boy, with evident paleness and distended abdomen. Past medical history was negative for previous gastrointestinal bleeding or episodes of cholangitis. An upper gastrointestinal endoscopy was performed, showing esophageal varices secondary to portal hypertension. Imaging studies revealed hepatosplenomegaly, alterations in liver echogenicity, and dilated saccular bile ducts affecting both liver lobes without observing any apparent obstruction, highly suggestive of CD. A liver biopsy revealed nodular liver tissue with marked fibrosis between nodules, which confirmed the presence of CHF. Both kidneys were increased in size, hyperechoic and with loss of corticomedullary differentiation. FLD commonly present with coexisting hepatobiliary and renal alterations. Therefore, starting at the time of initial diagnosis, all patients with ARPKD should be evaluated to detect liver abnormalities due to the high association. Despite the rarity of CS, especially in early childhood, the association between ARPKD and FLD is well documented. So if this clinical presentation arises, CS should be suspected.