In this case series descriptive study, we aimed at describing the clinical presentations and complications of congenital hepatic fibrosis. The study was conducted on patients attending the hepatology clinic Cairo University Pediatric Hospital (CUPH) from November 2011 to June 2013. Methods: Patient’s data were retrieved from patient’s files. Twenty nine patients with age range from 0.6 to 13.2 years were included; they were 19 males and 10 females. History of the presenting illness, clinical examination, and laboratory investigations were recorded. Ultrasound and upper endoscopic findings as well as liver biopsy findings were recorded as well. Diagnosis of congenital hepatic fibrosis depends on clinical, ultrasonographic findings as well as liver biopsy. Results: The main presenting symptoms were abdominal distension in 15 patients (51.7%), followed by hematemesis in 11 patients (37.9%). Failure to thrive was detected in the form of weight below 3rd percentile in 10 patients (38.5%). Hepatomegaly was the main clinical finding in the studied patients as it was detected in 24 patients (82.8%). Evidence of portal hypertension, manifested by splenomegaly was detected in 21 patients (72.4%) and esophageal varecies in 20 patients (69%) of patients. Recurrent cholangitis presenting with fever, jaundice in an already diagnosed CHF patients was detected in 3 patients (10.3%). Intra-hepatic biliary radical’s dilatation was found by ultrasound examination in 5 patients (17.2%). Increased kidney size was detected in 10 cases (34.5%), renal calcification was detected also in 10 cases (34.5%), renal cysts in 9 cases (31%), hyperechogenic kidney in 4 patients (13.8%), loss of cortico-medullary differentiation in 4 cases (13.8%), and renal stone was found in one case (3.4%). Unfortunately one of the studied patients died of hematemesis complicating esophageal varices. Conclusion: Portal hypertension was the main clinical presentation of congenital hepatic fibrosis. Renal involvement, the other spectrum of fibrocystic hepatorenal disease, is somewhat common in our cases.