International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. Pilonidal sinus is a chronic inflammatory condition associated with the penetration of hair fragments into the skin. The incidence of pilonidal sinus is 0.07%. We present the first reported case of pilonidal sinus occurring in preauricular area in a 22-year-old male. Case Report: A 22-year-old male presented with pain and pus discharge from the right preauricular area since two years. On local examination, a scar measuring 2 cm at right preauricular area was found with one external opening. Routine investigations were within normal limits. Complete excision of the scar with the tract was performed. Primary closure of the wound was done. Histopathological examination confirmed pilonidal sinus. Conclusion: Pilonidal disease is a complex condition that causes both discomfort and embarrassment to sufferers. The etiological origin of a pilonidal sinus is controversial. Although reported in several rare areas of the body other than sacrococcygeal area, to our knowledge, this is the first reported case in which pilonidal sinus occurs in preauricular area. Although pilonidal sinus is most frequently found in the sacrococcygeal region, it could occur in other area including preauricular area.