Pyomyositis is a primary purulent infection of skeletal muscles mostly described in tropical areas. Staphylococcus aureus is by far the main causative microorganisms. In temperate areas, pyomyositis more frequently affects male adults with immunodeficiency. Gram-negative bacilli pyomyositis are uncommon and occur mainly in this context of immunodeficiency. Only one case of Enterobacter cloacae pyomyositis has been reported so far. Chronic Granulomatous Disease (CGD) is a rare inherited immunodeficiency characterized by a loss of NADPH oxidase activity in phagocytic cells that favors infections due to catalase-positive microorganisms. Pyomyositis has only been described once as a complication of CGD. We report here a case of Enterobacter cloacae-associated pyomyositis of the left thigh in a patient with CGD, and review the literature on Gram-negative pyomyositis.