Immunoproliferative small intestinal disease in a 16-year-old boy presenting as severe malabsorption with excellent response to tetracycline treatment.

Immunoproliferative small intestinal disease (IPSID) is a rare lympho-proliferative disorder of the upper small intestine. It is considered a special form of MALT lymphoma with propensity to malignant transformation. This disorder is rare in pediatric literature. We report a case of IPSID in a 16-year-old boy with low-grade malignant transformation, presenting as severe malnutrition and a possible association with Helicobacter pylori. The patient responded well to an extended treatment with tetracycline and eradication of H. pylori.