Langerhans’ cell histiocytosis is an uncommon disease in children with varied clinical presentation. Multisystem form of this disorder usually affects organs like the bones, skin, liver, spleen, lungs, and the central nervous system. We describe here the clinical details of a 2-year-old girl with involvement of unusual sites like the parotid glands and the nails. This child also had multiple cystic lesions in the liver leading to a misdiagnosis of Caroli’s disease. Knowledge about the uncommon manifestations of this rare disorder helps in early diagnosis and treatment.

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Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disorder characterized by the presence of splenomegaly, pancytopenia, and the presence of abnormal B-lymphocytes with cytoplasmic projections giving the appearance of hairs projecting from their surface [1]. Unusual presentations of HCL include marked leukocytosis, spontaneous splenic rupture, bulky chest or abdominal lymphadenopathy, destructive bone lesions, and neurological complications including meningitis and peripheral nerve compression [2]. Ascites has been rarely reported as a presenting feature of hairy cell leukemia [3].A 52-year-old male presented with history of intermittent fever, fatigue, weight loss of 5 months' duration, and abdominal swelling of 2 weeks' duration. On examination he had massive ascites. He had no peripheral lymph node enlargement or clinical signs of chronic liver failure. Ultrasonography of the abdomen showed gross ascites, splenomegaly (15 cm), normal liver size and echotexture, and no lymph node enlargement. A computed tomography (CT) scan of the abdomen showed splenomegaly with multiple focal lesions, ascites, and mild right pleural effusion. His complete blood count was hemoglobin 9.3 g/dL, white blood cell (WBC) count 1500/dL, absolute neutrophil count 1200/dL, and platelets 64 000/dL. The differential count showed 30 neutrophils, 15 lymphocytes, two mononuclear cells, and three eosinophils out of 50 cells. Bone marrow aspirate showed 40% abnormal lymphoid cells with typical hairy cell morphology [ Figure 1(a)]. Bone marrow biopsy revealed 30-35% cellularity,

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