Pulmonary giant cell carcinoma is a rare subtype of sarcomatoid carcinoma. Pseudomyxoma peritonei (PMP) is a rare condition in which gelatinous material accumulates within the peritoneal cavity. It is believed PMP arises from a primary appendiceal mucinous neoplasm that perforates the gut, causing mucinous ascites. There are sporadic reports of PMP associated with neoplasms of other organs, rarely the lung. Here, we report on a 60-year-old woman with pulmonary giant cell carcinoma associated with PMP. She presented with progressive dyspnea and abdominal distention. Abdominal computed tomography revealed moderately dense ascites without an obvious mass. Chest computed tomography revealed a large, solitary right lower-lobe lung mass. She underwent transbronchial fine-needle aspiration of the mass, and was diagnosed with pulmonary giant cell carcinoma. The ascites showed scattered malignant cells in a background of mucin, confirming PMP. To our knowledge, this is the first report of pulmonary giant cell carcinoma associated with PMP.