Background and objectives: Many liver diseases are accompanied by jaundice. It is important to differentiate cholestatic jaundice from non-cholestatic jaundice. Cholestatic jaundice is most likely due to a pathological condition and the most common causes in infancy are neonatal hepatitis and biliary atresia early in life. Early diagnosis and treatment of childhood cholestasis can improve the prognosis of liver diseases by preventing complications of these disorders. Patients and methods: In this retrospective study, 122 infants under 3 months old with cholestasis at Nemazi Hospital (affiliated with Shiraz Medical University). Sciences) during the years 2001-2011 were studied. Demographic data, duration of jaundice, liver biopsy, and causes of cholestasis were recorded. Results: There were 76 men (62.3%) and 46 women (37.7%) with a mean age of 54.4 ± 23.7 days. The most common clinical finding was jaundice, observed in all patients (100%). Jaundice appeared between the first day and fifty-two days of age, with a mean age of 15.6 ± 16.1 days. Other findings included hepatomegaly in 92 patients (76.4%), clayey stools in 54 (44.3%), and splenomegaly in 29 patients (23.8%). In this study, the most common causes of cholestasis were biliary atresia (30 = 24.6%), idiopathic neonatal hepatitis (30 = 24.6%), and bile duct insufficiency (16 = 10.3). Conclusions: The results of this study showed that neonatal hepatitis and biliary atresia are the most common causes of childhood cholestasis in this region. This is a crucial warning which requires defining and implementing a regular system allowing faster diagnosis for the management of patients in the golden period. Keywords: Cholestasis, biliary atresia, neonatal hepatitis. Introductio...... middle of article ......most important causes of cholestasis in this region; therefore, greater attention to these can help doctors care for patients better and more efficiently. In general, the results of this study are consistent with those of previous studies and it seems that the most important factor in patient management is the definition and implementation of a regular system for faster diagnosis. Finally, the shortcomings of this study are its retrospective nature and the lack of patient survival examinations. Due to the sensitive nature of the disease and the need to raise awareness among general practitioners and pediatricians about the causes of cholestasis and its diagnosis, more precise studies are needed. It is suggested to pay more attention to genetic determination of metabolic disorders and provide facilities for diagnosis of these diseases in major medical care centers..