Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous malignancy with a low propensity for distant metastasis. When systemic dissemination occurs, it primarily targets the lungs, often secondary to a fibrosarcomatous transformation (FS-DFSP). We report the case of an 81-year-old Gambian male presenting with severe progressive dyspnea. He had a 14-year history of recurrent, disseminated cutaneous tumors across his limbs and torso, histologically confirmed as DFSP. Advanced thoracic imaging revealed a massive, space-occupying left intrathoracic mass causing complete lung collapse and a severe rightward mediastinal shift, alongside contralateral pulmonary nodules. To our knowledge, this represents the first documented report of advanced metastatic DFSP with extensive intrathoracic dissemination in West Africa. This case highlights the profound diagnostic challenges associated with the slow-growing nature of the tumor and the clinical difficulty less experienced physicians face in linking indolent dermatological conditions to internal organ affection. Furthermore, it underscores the logistical hurdles of managing complex soft-tissue sarcomas in resource-limited environments where specialized diagnostic validation and targeted therapies are unavailable.