Morphea is an autoimmune condition characterized by excessive accumulation of collagen in the skin and underlying tissues. Lichen sclerosus (LS) is another connective tissue disease for which an autoimmune cause has been proposed, given the high association with other autoimmune diseases. The coexistence of morphea and lichen sclerosus has been sometimes reported in the literature, and is suggestive of a common pathogenic background between the two diseases. Among various types of morphea, generalized morphea has been associated with an increased rate of autoimmune disease, including LS. We report a case of extragenital LS during the progression of linear morphea into generalized morphea.