Children acquire blastomycosis, with rare exceptions, through the respiratory route. Nearly half of those who are infected may be asymptomatic. Cough is the most common symptom and is usually without sputum production, and hemoptysis is not noted. Other symptoms are chest pain (described as tightness or pain when breathing), weight loss, night sweats, and loss of appetite. The severity of illness is variable and may simulate an upper respiratory infection, bronchitis, pleuritis, or pneumonia. As in adults, an overwhelming infection may cause respiratory failure even in immunocompetent children and in immunocompromised children who live in or travel to endemic areas are susceptible to infection. Some reports based on consecutive cases note extrapulmonary dissemination commonly in children, whereas dissemination is rarely noted in outbreak cases. Chronicity of the disease favors extrapulmonary dissemination. Chest radiograph patterns are alveolar infiltrates, consolidation, and nodule(s), and these may be accompanied by cavitation. Diagnosis is suspected when the symptoms that mimic common respiratory infections persist for more than 2 weeks and by a history of residence or travel to an endemic area. Chest radiographic findings of nodule(s) or cavitation further increase the suspicion. Confirmation of diagnosis is by microscopic examination and culture of sputum. When expectorated sputum is unavailable, bronchoscopy with lavage and biopsy or percutaneous needle biopsy of lung is the appropriate next step. Disease that is progressive or severe or disseminated to other organs should be treated. Amphotericin B is effective and results in excellent cure rates. Experience using oral azoles is limited in children.