Clinico-pathological case report of a 11 year-old girl who successively presented persistent abdominal pain, pneumoperitoneum, repeated intestinal hemorrhages, superficial intestinal ulcerations, first localized on the jejunum and later extended to the all gut. After a course of 13 months, the child died from diffuse and repeated hemorrhages. The morphologic studies revealed a diffuse and homogeneous cellular infiltrate made of large round "lymphomatous" cells scattered within the lamina propria and submucosa. Initially this superficial cellular infiltrate was overshadowed by accompanying inflammatory cells and was not recognized as a tumoral process. Later on the dissemination of abnormal cells to the entire ileon, mesenteric lymph nodes, spleen and liver and the cellular appearance confirmed its neoplastic nature and allowed to consider this process as a malignant histiocytosis of the intestine as described by Isaacson and Wright. This case seems to be the first case reported in childhood.
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