&NA;A nine‐year old boy with primary hypogammaglobulinemia and recurrent colonic strictures who developed malakoplakia of the colon is reported on. Such an association has not been reported previously. The development of colonic strictures is not considered a gastrointestinal manifestation of hypogammaglobulinemia. Malakoplakia of the colon is a rare disease, with 26 cases being reported to date. Only five children under age 13 have been reported with colonic malakoplakia and none with hypogammaglobulinemia. Hypogammaglobulinemia should be added to the growing list of conditions predisposing to the development of malakoplakia.