A previously well 59-year-old man presented with paracoccidioidomycosis, more than 15 years after leaving South America. He failed to respond to conventional therapies, first with oral itraconazole and then with amphotericin B plus sulfadiazine, and eventually died of recurrent arterial emboli possibly due to paracoccidioidomycotic aortitis. This patient's presentation demonstrates the difficulties that may be encountered in diagnosing and managing this disease. Paracoccidioidomycosis should be suspected in patients with an appropriate travel history who experience weight loss and have pulmonary, mucosal, and cutaneous lesions. This article comprehensively reviews the literature, with emphasis on epidemiology, clinical presentation, diagnosis, and therapy with imidazole antifungal medications.