Disabling pansclerotic morphea of childhood (DPMC) is an aggressive form of cutaneous scleroderma that involves all layers of the skin, extending through the dermis and subcutaneous tissues to involve muscle, tendon, and even bone. As DPMC is extremely rare, its association with skin squamous cell carcinoma (SCC) is rarer still. To our knowledge there are only two cases of SCC in patients with DPMC that developed within areas of chronic skin ulceration. We report the first case of lower lip squamous cell carcinoma arising in a young woman with DPMC and discuss the carcinogenic pathway that may have led to its occurrence.