Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians, pediatricians, and dermatologists. It affects both sexes with a lower incidence in men than in women. Autoimmune factors, infections, and genetic disposition are implicated in its pathogenesis. The classic clinical picture is of atrophic white plaques in the anogenital region. Pruritus, soreness, difficulty retracting the foreskin, and obstructive urinary symptoms are frequent complaints. Early histological confirmation by biopsy is recommended. The disorder is usually highly responsive to ultrapotent topical steroids. This may help arrest or delay the progressive nature of this disorder. Surgical treatment may be needed in cases of disease progression despite medical therapy. Circumcision is a successful treatment, if LS is limited to the glans and foreskin. In cases of meatal stenosis meatoplasty and in cases of urethral involvement one- or two-stage oral graft urethroplasty is necessary.