Spontaneous hypoglycaemia is usually caused by an insulin-producing islet-cell tumour of the pancreas. Rarely, it can be caused by non-islet cell tumours. Most of the tumours are of mesenchymal type, large, and slowly growing. One representative is haemangiopericytoma (HAP). The present report describes a case of a large recurrent retroperitoneal HAP associated with severe hypoglycaemia. Blood serum insulin and proinsulin concentrations were low. By means of acid-gel chromatography and dot-blot techniques, an increased amount of a high-molecular-weight IGF-2 peptide was found. By using antigen retrieval procedures, IGF-2-immunoreactive tumour cells were found in specimens of the recent tumour recurrence-but not in the original. When the in situ hybridization technique was used it could be shown that IGF-2 mRNA labelling had already occurred in the original tumour specimen, 11 years before the onset of hypoglycaemic symptoms. These observations confirm the hypothesized hypoglycaemic effects of high-molecular-weight (HMW) IGF-2, but also point to the presence of a prolonged compensation of this effect. A literature review, based on 17 similar cases of haemangiopericytoma with hypoglycaemia, is presented. Our observation and findings in the literature review support the idea that non-islet-cell tumour hypoglycaemia is caused by an overproduction of a HMW IGF-2 peptide. The insulin-like effect is mediated via non-specific binding to the insulin receptors. To anticipate patients at risk of developing this kind of hypoglycaemia, the histopathological investigation should include not only immunohistochemical analyses of the presence of IGF-2 peptide, but also in situ hybridization of the IGF-2 mRNA expression.