Objective:

To describe a case of Langerhans cell histiocytosis in a pediatric patient.

Method:

A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis.

Results:

The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis.

Conclusion:

Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.