Malakoplakia is a rare pseudotumoral inflammatory disease known to affect immunocompromised subjects, mainly with a history of recurrent Escherichia coli infection. The urinary tract is the most frequent site of the disease, although all organs can be involved. In the present article, we report a case of malakoplakia of the caecum, that developed in a 52-year-old man, who had received a kidney transplant 9 years before and had a history of recurrent E. coli urinary tract infections. Malakoplakia presented as acute intestinal perforation, and, despite aggressive surgical and medical management, disease progressed toward a fatal outcome due to sepsis and multiple organ failure 9 months later. A defect in the macrophagic activity was demonstrated.