Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract. The presence of malignant lesions arising in the diverticulum is very rare, the most common malignant lesion being sarcoma, followed by carcinoid tumors, and less frequently by adenocarcinomas. We present the case of an 86-year-old man who developed acute abdomen. Surgery revealed a perforated Meckel's diverticulum. Histology identified a poorly-differentiated adenocarcinoma arising in Meckel's diverticulum. We provide a review of the literature.