The Chediak-Higashi syndrome (CHS) is a rare disease and is very often observed in a single family. In Venezuelan Los Andes area, its appearance is not uncommon. One of the peculiar aspects of CHS patients is the color, texture and structure of the hairs. We have, therefore, investigated structure, morphology and composition of hairs and compared those properties with that of normal human subject grown in the same geographical region. A few hairs were cut along its length and their fluorescence was investigated. For CHS patients, dark patches were observed. Investigations of chemical compositions of these regions were carried out of using Energy Dispersive Analysis and it was found the presence of Na, K and Ca ions. Fluorescence spectral studies were carried out at room temperature. Observed spectra reveal two peaks, however, those were not useful for positive identification of CHS.