We report an 8-year-old boy with acute leukemia who developed ecthyma gangrenosum secondary to infection with Exserohilum spp., a rare cause of human disease. The skin, paranasal sinuses and lungs were involved. To the best of our knowledge, this is the first report of ecthyma gangrenosum caused by Exserohilum spp. This case emphasizes the importance of prompt skin biopsy for early diagnosis and treatment of this life-threatening infection.