A progressive scarring alopecia of the central scalp is commonly seen in young to middle-aged females of African descent. It usually starts at the vertex or mid top of the scalp and gradually spreads centrifugally, hence, the unifying term of central centrifugal cicatricial alopecia. The clinical pattern is suggestive of female pattern alopecia, but a lack of follicular pores indicative of scarring is present. It can progress for years before slowly burning out. The etiology is unknown but genetic factors may be important. It is often associated with a history of traumatic hairstyling involving heat, traction, and chemicals. However, most patients of African descent without this disorder have similar styling habits. Nonetheless, avoidance of physical and chemical trauma to the scalp hair, the use of suitable shampoos and conditioners, and the encouragement of natural hairstyles may be helpful. Any infection should be treated. Topical or intralesional corticosteroids and systemic antibiotics may be useful and topical minoxidil should be tried with the hope of preventing further scarring and encouraging regrowth of recovering follicles. Current research into the etiology of this disorder will help to foster much-needed clinical trials of therapeutic agents.