Ebers Papyrus of Ancient Egypt described a swelling containing hair possibly a pilonidal sinus that is to be treated with the knife treatment. There are two main theories that have been established to explain the occurrence of sacrococcygeal pilonidal sinus disease and yet none is accepted or supported, congenital and acquired theories. Hair is forced into the depth of the natal cleft and penetrates into the subcutaneous tissues through the dilated hair follicles. A foreign-body reaction develops, which is accompanied by infection and the subsequent development of the primary pilonidal sinus. Pilonidal sinus disease has three different clinical presentations: asymptomatic form, acute abscess form, and chronic disease form. A spectrum of treatment of chronic pilonidal sinus disease varies from nonoperative treatment to complex advancement flap operation. Choosing the appropriate surgical technique for pilonidal sinus disease should be tailored and individualized according to the patient’s satisfaction and the presentation of the disease itself.