Adhesion molecules work for leukocytes to infiltrate to the site of inflammation. Deficiencies of these molecules can disturb neutrophil migration and thus inflammation. This group of illnesses is named leukocyte adhesion deficiency (LAD) syndromes. Mastoiditis is a very rare and unexpected complication. CD 18 count is the prognostic factor and might affect the treatment modalities. We report the case of a six-year-old girl with LAD type 1 who presented with chronic mastoiditis and the treatment of this unusual presentation.Keywords: Chronic middle ear disease, leukocyte adhesion deficiency, pediatric ear disorders, pediatric mastoiditis, primary immunodeficiency syndromes.