Sarcoidosis-induced sclerosing cholangitis is an exceptionally rare manifestation of multisystem sarcoidosis, often mimicking primary sclerosing cholangitis (PSC). Hepatic involvement occurs in 50–70% of patients, though only 10–15% develop symptoms.Biliary tract involvement is exceedingly uncommon, with fewer than 30 cases reported.A 28-year-old female presented with insidious cholestatic symptoms progressive pruritus, dark urine, and 10 kg weight loss over six months. Laboratory evaluation revealed markedly elevated alkaline phosphatase (1143 U/L) and γ-glutamyl transferase (757 U/L), with total bilirubin 3.8 mg/dL and direct 2.0 mg/dL. Mild normocytic anemia was present. The pattern reflected a cholestatic injury with an ALP-to-ALT ratio of\ 15:1.Ultrasound demonstrated proximal common bile duct stricturing with intrahepatic biliary dilatation. MRCP showed multifocal biliary strictures with loss of flow enhancement and splenic lesions.PET-CT revealed hypermetabolic lymphadenopathy (cervical, hilar, abdominal),pulmonary ground-glass opacities, and splenic involvement—suggestive of multisystem granulomatous disease.Histopathology from splenic FNAC and cervical lymph node biopsy revealed non-caseating granulomas with multinucleated giant cells, no necrosis. Serum ACE levels were markedly elevated, supporting sarcoidosis.The collective findings confirmed sarcoidosis-induced secondary sclerosing cholangitis after exclusion of infectious and autoimmune causes.Systemic corticosteroid therapy was initiated, resulting in rapid improvement: bilirubin reduced to1.3 mg/dL,ALP to 471 U/L, with symptomatic relief and partial resolution of biliary strictures on follow-up MRCP.Pathophysiologically, granulomatous infiltration of portal tracts causes inflammatory bile duct stricturing.Unlike PSC, these lesions are potentially reversible with immunosuppression.Diagnostic clues favoring sarcoidosis include multisystem involvement, elevated ACE, absence of p-ANCA/AMA, and steroid responsiveness.Conclusion:Sarcoidosis-induced sclerosing cholangitis, though rare, is a treatable cause of cholestatic liver disease. Comprehensive evaluation for systemic sarcoidosis should be performed in all patients with atypical sclerosing cholangitis. Prompt corticosteroid therapy can yield substantial clinical, biochemical,and radiologicalmprovement.KeyMessages Evaluate for extrahepatic granulomatous disease in sclerosing cholangitis. Elevated ACE and multisystem involvement suggest sarcoidosis.Exclude infectious and autoimmune causes before diagnosing PSC.Early immunosuppressive therapy offers favorable, reversible outcomes.