Central centrifugal cicatricial alopecia (CCCA) is a common and progressive form of lymphocyte predominant scarring alopecia which impacts negatively on the quality of life of those affected. It is seen more commonly in women of African descent with prevalence ranging from 2.7% to 5.7%. Current postulates include genetic inheritance, with traction inducing hairstyling practices and hair chemicals as aggravating factors. Histology reveals a perifollicular lymphocytic inflammation of the lower infundibulum, premature desquamation of the inner root sheath, and fibrous connective tissue. Treatment remains challenging and is directed at suppressing and preventing the inflammation, thus aborting scarring, with surgical intervention reserved for those who have stable disease or absence of histological inflammation. Future research with more patient numbers, focusing on the genetics of CCCA may prove useful in order to fully understand the etiology, thus providing more effective treatments for CCCA.