Progressive familial intrahepatic cholestasis (PFIC) is a rare group of inherited liver disorders affecting newborns and young children, leading to progressive liver disease and cirrhosis. With an estimated frequency of 1 per 50,000 to 1 per 100,000 births, PFIC is classified into six types, with PFIC-1 and PFIC-2 being the most common. This case report presents a 42-day-old male infant with jaundice and abnormal liver parameters, diagnosed with PFIC type 2 through clinical and investigative evaluation. The infant exhibited persistent jaundice, high-colored urine, and elevated liver function tests, including total serum bilirubin, SGOT, SGPT, ALP, and serum bile acids. A liver biopsy revealed giant cell hepatitis with mild portal fibrosis and bile duct proliferation, while a HIDA scan ruled out biliary atresia. Treatment included ursodeoxycholic acid, phenobarbitone, and fat-soluble vitamin supplementation, with a plan for liver transplantation. PFIC is a distressing cholestatic liver disorder leading to end-stage liver disease. Diagnosis involves clinical, laboratory, and genetic evaluations. PFIC types 1 and 2 present early in infancy, while PFIC type 3 manifests later. Extrahepatic symptoms and elevated GGT levels aid in differentiating PFIC types. PFIC management is challenging, with medical therapies offering limited benefits and surgical options such as liver transplantation providing effective relief. Early diagnosis and intervention, including biliary diversion and transplantation, are crucial to prevent significant morbidity and mortality from end-stage liver disease.