A 6-year-old boy reported along with his mother, to the dental outpatient department of Government Dental College, Hyderabad, India with the chief complain of loosening of lower back teeth since two months. Patient's mother gives the history of gradual increase in mobility of the teeth with subsequent loss of left lower back tooth about 10 d ago. Mother gave no history of trauma, and no association of pain and swelling. Mother further revealed slow growth of the child when compared to his classmates. She also gave history of frequent urination. In the past medical history, patient's mother gives history of right ear infection about three years ago. No relevant dental history.On general examination, patient appeared tensed but answered all the questions coherently, and was poorly built with reduced height (95 cm) and weight (12 kg) when compared to normal boy of same age (height = 116 cm; weight = 21 kg). On extraoral examination, the patient had brachycephalic skull, with frontal bossing, slight hypertelorism, exophthalmic left eye with pallor in relation to palpebral conjunctiva [ Table/ Keywords: Histiocytosis, Hand-schuller-christian disease, Langerhan cell appeared slightly reddish in colour. No visible pulsations with normal surrounding mucosa. On palpation, all inspector findings with respect to number, site and shape was confirmed. The swelling was tender to touch and with firm consistency. The swelling was neither reducible nor compressible and was fixed to the underlying structures. No palpable pulsations.On intraoral examination, the gingiva was erythematous, oedematous and with loss of stippling in relation to 53, 54, 55, 63, 64, 65, 73, 74, 83, 84, 85. Gingival recession was present in relation to 75, 84, 85 exposing the entire roots. 74 were missing. Grade I mobility was present with 54, 55, 64, 65, 71, 72, 73, 75, 81, 82 83, 84, 85. Based on the case history and clinical examination, a provisional diagnosis of Hand-schuller-christian disease was given. The differential diagnosis of Down's syndrome, cyclic neutropaenia, hypophosphatasia and acute leukaemia's was given, as the entire above are associated with young age, loosening of deciduous dentition and may be associated with stunted growth and hence were considered.The peripheral blood smear revealed hypochromic microcytic anaemia. The complete urine examination revealed low specific gravity (1002) and daily urine output was about 3.2 litres per day. Lateral skull radiograph revealed multiple well defined punched out radiolucencies [Table/ Fig-3], while the orthopantomograph revealed irregular bone destruction in relation to 73, 34, 75, 35, 84, 85 and