A 9-year-old boy of Moldovan origin presented to the Emergency Room with jaundice and pruritus persisting for two months. His medical history revealed recurrent, self-limiting episodes and a recent hospitalization in Moldova for hyperbilirubinemia treated with plasmapheresis. Laboratory tests showed hyperbilirubinemia, elevated transaminases and prolonged INR; abdominal ultrasound revealed hepatomegaly without focal lesions. Following treatment with vitamin K and ursodeoxycholic acid, the patient was admitted for further evaluation. Liver biopsy showed non-mechanical cholestasis with bilirubinostasis and initial ductopenia. Whole-exome sequencing (WES TRIO) confirmed a pathogenic mutation in the ABCB11 gene, consistent with progressive familial intrahepatic cholestasis type 2 (PFIC2). Benign recurrent intrahepatic cholestasis (BRIC) is an inherited disorder characterized by intermittent episodes of intrahepatic cholestasis; it manifests with jaundice and pruritus of variable duration, alternating with asymptomatic periods. Onset typically occurs in childhood or adolescence; triggering factors may include viral infections, oral contraceptive use and pregnancy. Diagnosis relies on clinical history, biochemical investigations and liver biopsy. The prognosis is generally favourable, although progression to PFIC and cirrhosis may occur