Penile lichen sclerosis is a longstanding inflammatory disease of the skin with a controversial aetiology. Penile lichen sclerosis (PLS) is a growing, inflammatory dermatitis of the anogenital region, which involves the meatus, prepuce, penile shaft, and glans penis. Although the accurate aetiology of PLS is contentious, multiple factors including genetics, autoimmunity, infections of human papillomavirus, hepatitis C, Epstein-Barr virus, risk factors (hormonal and trauma), etc., can be considered to be a part of the etiopathogenesis of PLS. The initial clinical presentations of penile lichen sclerosis are white plaques, atrophied skin, erythema, erosions, and sclerosis in the anogenital region. When the disease advances, the following can occur, including meatal constraints, telangiectasia, petechiae, soreness, papular lesions, tightness of the foreskin, difficulties in passing urine, itching, tenderness on erections, pain, cracking, bleeding, redness, rashes, tightness at frenulum, and dysuria. This disease has a dangerous course of action and if untreated it may be linked with severe urologic and sexual morbidities. PLS is usually treated with medical and surgical interventions like topical or intralesional steroids and circumcision. The role of circumcision is very critical in the course of action and prognosis of PLS, and its treatment is dependent on the stage of the disease. This review brings up the knowledge regarding epidemiology, etiopathology, clinical presentation, and management of PLS with an emphasis on the role of circumcision.