Objective: Fibrocystic liver disease (FLD) is a multisystemic disease that can be seen in a wide age range from intrauterine period to adolescent age. The aim of study is to evaluate the presenting symptoms, clinical-laboratory findings, treatment modality and results of the patients with FLD . Material and Methods: The demographic features, clinical-laboratory findings, treatment modality and results of patients with FLD followed up in our clinic between January 2008 and December 2019 were recorded retrospectively. Results: A total of 39 patients (56.4% male, median age; 53m years, age range: 10 days-16.8 years) were evaluated. Eight patients (20.5%) had Caroli’s disease (CD), 16 patients had congenital hepatic fibrosis (CHF) (41%), and 15 had choledochal cysts. The most common presenting symptoms were jaundice (n=8, 20.5%), chronic abdominal pain (n=6, 15.4%) and splenomegaly (n=4, 10.3%). Eight patients were detected after renal cyst detection and screening programme (20.5%), seven patients during intrauterine period (17.9%), and two patients incidentally (5.1%). PKHD1 gene mutation was deteceted in six patients with autosomal recessive polycystic kidney disease (ARPKD). Eighteen patients underwent surgical operation (46.2%, liver transplantation, left lobe segmental hepatectomy, mesocaval shunt, kidney transplantation, cystectomy). 25 patients (64.1%) had extrahepatic involvement [ ARPKD (n=18), mental motor retardation (n=2, methochromatic leukodystrophy, Arnold Chiari malformation in each one), nephrocalcinosis (n=1), juvenile nephronophytosis (n=1) acute pancreatitis (n=1), pulmonary hypoplasia + metacarpal distal phalanx hypoplasia (n=1) and medullary sponge kidney+pineal cyst (n=1)]. During the follow up of 39 patients; six patients had portal hypertension, five had chronic renal failure (12.8%), four had compensated chronic liver disease (10.3%) and one had recurrent cholangitis attacks (2.6%). Two patients underwent liver transplantation due to decompensated cirrhosis, and one patient underwent kidney transplantation due to end-stage renal failure. Conclusion: Early diagnosis, regular follow-up and treatment are important in patients with FLD because of the high risk of morbidity and complications.