Fatal pneumococcal disease occurred in a young woman with ulcerative colitis that had been quiescent for ten years. Howell-Jolly bodies were present in the peripheral blood smear. Severe atrophy of the spleen (weight 7 g) was discovered at autopsy. The evidence that hyposplenism is associated with inflammatory bowel disease is reviewed and it is suggested that these patients should undergo ultrasound examination and examination of blood smear for detection of functional hyposplenism. We conclude that pneumococcal vaccination should be considered for all patients with inflammatory bowel disease.