Coup de Sabre is a subset of localized linear scleroderma disease influenced by autoimmune, vascular, and genetic factors. The disease often follows an active, relapsing, and remitting course causing the development of permanent sequelae, such as tissue atrophy, pigment alterations, and bony deformities. Treatment strategies have ranged from surgical to injectable and medical management approaches with varying success. Although more common in pediatric patients, Coup de Sabre remains rare, with limited literature available on disease course and treatment outcomes, particularly in the sub-Saharan African population. Furthermore, the existence of autoimmune conditions occurring simultaneously with HIV infection presents an interesting discussion of the interplay between antiretroviral medication, immune dysregulation, and autoimmunity. This case report describes an 11-year-old HIV-positive male in Lilongwe, Malawi with mixed subtype of localized scleroderma manifesting most prominently as Coup de Sabre that remained undiagnosed for 7 years.