Congenital occipital encephalocele with Dabska tumor: report of an unusual case.

Encephaloceles arise from developmental defects in neural tube formation. These lesions contain brain and meninges which herniate through a defect in the skull. These may present as isolated malformations or rarely be associated with brain tumors. We hereby discuss a case of an unusual association of an occipital encephalocele with papillary intralymphatic angioendothelioma or Dabska tumor arising from the sac itself. The patient underwent resection of the herniated brain tissue with repair and closure of the dural defect. Histopathological examination revealed evidence of Dabska tumor from the sac. This is the first case report of the association of an occipital encephalocele with a rare vascular tumor, i.e. papillary intralymphatic angioendothelioma.