A case of chronic omphalitis present for birth in a 4-month-old girl is presented. The biopsy of the bud-like lesion failed to reveal a local malformation or remnants of umbilical cord but showed a common loose edematous tissue in which the inflammatory cells appeated remarkably scanty. The contrast existing between this poorly cellular local infiltrate and the high level of peripheral blood leucocytes (over 30,000/microliters) was in fact the most striking feature that allowed to evoke the diagnosis of Deficiency Leucocyte Adhesion molecules. Immunocytochemical investigations using anti CD11a, CD11b and CD18 monoclonal antibodies on fresh tissue or, better, peripheral leucocytes are necessary to confirm the diagnosis of this uncommon immunological autosomic recessive inherited disorder.