The chest wall chondrosarcoma (CWC) is a rare slowly growing primary tumor of the chest wall with an incidence of <0.5 per million person-years. We present the case of a giant CWC that caused a mass effect on the mediastinum, heart, and lung. Large tumors with thoracic structures compression may be life threatening, and its resection and subsequent chest wall reconstruction represent a significant multidisciplinary surgical challenge. In this case, despite the large tumor dimensions, the preoperative planning—sparing key reconstructive options without compromising the tumor resection—allowed a complete en bloc tumor excision of a grade III chondrosarcoma with negative histologic margins. Successful reconstruction of the large full-thickness chest wall defect, with a latissimus dorsi muscle flap and methyl methacrylate incorporated into a polypropylene mesh in a sandwich fashion, was accomplished. Patient recovery was uneventful with good functional and aesthetic outcomes, and no evidence of recurrence at 1.5 years follow-up. This case report illustrates the main clinical, radiological, and histologic features of a CWC while discussing the surgical goals and highlighting the principles for chest wall reconstruction following extensive resection of a large and rare entity.