Solitary fibrous tumours (SFT) of the pleura are uncommon and are incidental findings or discovered in patients with non-specific respiratory symptoms. We report a case of a 74 year old man diagnosed with a mesenchymal pleural neoplasm, associated with typical hypertrophic osteoarthropathy, referred to as Pierre-Marie-Bamberg syndrome. As reported in the literature, complete surgical resection is the gold standard for treatment of such lesions and recurrences. Radiotherapy and chemotherapy are of limited value in the curative treatment of pleural SFT. In our case surgical excision of the mass was performed. After a disease-free period of 3 years a second intervention was necessary because of recurrence. Until now our patient is free of complaints and no signs of reappearance were noted. Based on our experience and on literature findings we would like to underline the importance of regular long-term follow-up because of the substantial risk of recurrence.