We report the second case of a malignant paraganglioma of the stomach in a 56-year-old female patient. However, our case is the first investigated by immunohistochemistry and electron-microscopy. The tumour was characterized immunohistochemically by the presence of neurofilament protein, glial fibrillary acidic protein, S-100 protein, neuron-specific enolase, chromogranin A, ACTH, leu-enkephalin and vasoactive intestinal polypeptide, and ultrastructurally by demonstration of neurosecretory granules and paranuclear intermediate filament whorls. Despite massive metastatic spread in the abdominal cavity, the patient is still alive 4 years after initial diagnosis.