Background and aims Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause with manifestations ranging from isolated granulomatous lesions to life-threatening multi-system organ involvement.

In this study we aimed to evaluate the characteristics, diagnosis, treatment modalities and prognosis of LCH.

Methods We conducted a retrospective study of all cases of LCH in paediatrics department of Hedi Chaker University Hospital in Sfax during a period of 16 years (1997–2013) Epidemiologic, clinical, radiological, diagnostic and therapeutic variables were collected.

Results We collected 11 cases of LCH. The average age at diagnosis was 3 years 4 months. The patients’ presenting symptoms were: exophtalmia (3 cases), polyuropolydispsic syndrome (3 cases), prolonged fever (2 cases), lymphadenopathy (5 cases). Laboratory tests showed diabetes insipidus (3 cases) and bicytopenia (1 case). The diagnosis was confirmed by histopathologic examination in all cases.

Bone was the most frequently affected organ (9 cases) followed by skin (19.2%). Initially, 4 patients had single-system involvement (SS), 3 with mulisystem (MS) disease without risk organ involvement (MS-RO), and 4 multisystem disease with risk organ involvement (MS-RO). Chemotherapy based on vinblastine with corticosteroids was used in 4 patients who had MS-RO form. The outcome was favourable in 6 cases.

Conclusions Langerhans cell histiocytosis is a rare and heterogeneous disease. Multisystem disease with risk organ involvement justify the use of many drugs.