The Langerhans Cell Histiocytosis is a group of diseases with a single or multisystem involvement, it´s clinic and complications vary depending on the tissue affected and the extent of the injury. The case of a 2 ½ years old female patient was presented, with a mass on the skull in the left parietal area of 22 days, 6-7 cm long, painful, deforms facial image and loss of appetite. The CT scan showed a fracture of the parietal bone, increase of homogeneous solid volume without calcification. The treatment was a tumor resection, craniotomy and cranioplasty, the histopathological study showed immunohistochemically cells S100, CD1a and CD68 positive

Histiocytosis; eosinophilic granuloma; Histiocytosis X; Langerhans cell histiocytosis; Langerhans cells; lytic lesions; biopsy; immunohistochemistry; CD1a; S100; CD 68