Purpose: Langerhans Cell Histiocytosis (LCH) is a rare disease characterised by clonal proliferation of histiocytes. We report 3 cases of LCH presenting with jaundice. Methods: Retrospective analysis was done of cases presenting as jaundice over last 2 years and diagnosed to be LCH. Results: Case #1- A 19 months child presented with jaundice, itching, dark urine, abdominal distension and decreased appetite for 1 month with fever for 1 week at the onset of symptoms. On examination child was malnourished (wt 8.7 Kg < 5th percentile, ht 83 cm, 25th percentile), had icterus and hepatomegaly (liver 7 cm). Investigations revealed deranged LFT (Bilirubin t/d 4.7/3.3, ALT 57 IU/dl, γGT 491 mg/dl, S. albumin 2.3 g/dl, triglycerides 266 mg/dl). Viral markers were negative. Liver biopsy and MRI were suggestive of sclerosing cholangitis. History of rash (apparently seborrheic) at 1 month of age with spontaneous remission raised the suspicion of LCH. X-ray skull showed osteolytic lesions in skull. Chest x-ray showed cystic changes, confirmed on CT scan. Liver and bone marrow biopsies were conclusive for LCH. Case #2- A 15 months child, presented with intermittent jaundice clay coloured stools abdominal distension and failure to thrive for 6 months and gum hypertrophy with bleeding for last 10 days. On examination child was malnourished (weight 8.8 Kg, height 72 cm; both < 3rd percentile), had icterus, pallor, clubbing, gum hypertrophy with bleeding and hepatosplenomegaly (liver 5 cm, spleen 4 cm). Investigations revealed Hb 8.6 gm/dl, deranged LFT (Bil t/d 5.4/3.5, ALT 50 IU/dl, S.albumin 2.1 gm/dl, γGT 176 mg/dl). Bone marrow was hypercellular with no abnormal cell and liver biopsy done outside at 11 months age suggested cholestasis with periportal fibrosis and bile ductular proliferation. MRCP showed no IHBR dilatation thin right and left hepatic ducts with 2 mm CBD and rudimentary gall bladder. Seborrheic dermatitis of scalp and history of recurrent ear discharge alarmed the diagnosis of LCH. Bone scan showed osteolytic lesions in skull, maxillary alveolus and radius. Liver, skin and bone marrow biopsy were conclusive for LCH. Case #3 – A 29 months child, presented with skin rash for 6 months, constipation and failure to thrive for 4 months, pallor for 10 days. On examination child was malnourished (weight 9 Kg and height 81 cm; both < 3rd percentile), had icterus, pallor, skin rash and hepatomegaly (liver 5 cm). Investigations revealed Hb 9.8 gm/dl, deranged LFT (Bil t/d 5.9/4.1, ALT 164 IU/dl, S.albumin 2.1 gm/dl, γGT 725 mg/dl). Bone scan was normal. A liver, skin and bone marrow biopsy were conclusive for LCH. Conclusion: Jaundice, cholestasis and sclerosing cholangitis are rare manifestations of LCH and need high index of suspicion for diagnosis.