Carcinoid tumours are relatively rare neoplasms that often present as diagnostic dilemmas due to obscure or non-specific symptomatology. The ability of carcinoid tumours to cause clinical symptoms by secretion of hormones or biogenic amines is best recognised in the form of the carcinoid syndrome. Although generally slow growing, a significant proportion demonstrates aggressive tumour growth and may be difficult to manage. Ten percent of carcinoids are part of the spectrum of multiple endocrine neoplasia type 1, which should be considered in the investigation and management of these patients. A further 10% of carcinoid tumours are associated with other noncarcinoid tumours of various histological types. This review discusses recent improvements in biochemical diagnosis with the introduction of plasma chromogranin A, and puts into context the use of the imaging modalities, including Indium-111 Octreotide scintigraphy, and newer developing techniques, such as positron emission tomography. The therapeutic options are reviewed, including the use of somatostatin analogues as the treatment of choice in the control of hormone-mediated symptoms, the role of surgery, the use of chemotherapy, biotherapy using interferon, and the exciting new field of receptor-targeted therapy. In addition, the challenging interventional management of liver metastases is discussed, including the role of hepatic-artery embolisation and chemo-embolisation, radiofrequency ablation and the place of orthotopic liver transplantation in selected patients. We conclude that with the increasing number of investigative procedures and therapeutic options available to diagnose and treat carcinoid tumours, a multidisciplinary approach is needed. Furthermore, additional scientific research and controlled clinical trials are needed to determine the efficacy of the many treatment options, which for these rare tumours can only be achieved by collaboration.