Effective systemic therapy for advanced pseudomyxoma peritonei (PMP) is the focus of investigation. We describe a case of PMP arising from an adenoma of the appendix in a 58-year-old man. First, the patient underwent explorative laparotomy with ileocoecal resection, but without possibility of major tumour debulking due to adhesive gross tumour masses. Subsequently, six cycles of Folfox IV chemotherapy were administered, without response, but with severe side effects. Upon progressive disease, a combination of bevacizumab and capecitabine led to a long-term stabilization of disease and obvious improvement of performance status. Our case suggests that modulation of tumour microenvironment and angiogenesis by bevacizumab, potentially augmented by monochemotherapy, may be beneficial in borderline tumours such as pseudomyxoma peritonei. © 2009 Springer.