Cholestatic jaundice, defined as conjugated bilirubin levels higher than 1 mg/dl, is a bile formation and excretion disturbance. Its incidence is estimated at 1 case in 2500 live births, being 100-200 times higher in preterm infants less than 28 weeks gestational age. It occurs in biliary atresia, infectious diseases, endocrine and inherited metabolic diseases, Alagille Syndrome, preterm and intrauterine growth-restricted newborns, lack of enteral feeding, and is more frequent in male gender. It is a frequent complication of parenteral nutrition. In preterm and small for gestational age (SGA) infants, the etiology of cholestasis is multifactorial. Early diagnosis enables early therapeutic intervention. We report the case of an SGA male preterm infant. He was born by C-section for fetal distress at 30 weeks gestational age with an 800 g birth weight. He developed mild respiratory distress, hypoglycemia, meconium ileus, and early cholestasis. Complex hematological, serological, and immunological tests were carried out; ultrasound evaluations were performed. The final diagnosis was cholestasis of multifactorial etiology in a preterm infant with severe intrauterine growth restriction (IUGR). Treatment with ursodeoxycholic acid was started. Three months later, the transaminases, bilirubin, gammaglutamyl transferase (GGT), and alkaline phosphatase (ALP) triglycerides returned to normal. Conclusion. Cholestasis in SGA infants is a severe condition potentially associated with life-threatening complications, requiring complex diagnostic evaluation.