The incidence of systemic fungal infections increased during the last two decades. Rare fungi, such as Mucor, Fusarium and Paecilomyces, are emerging as causes of systemic fungal infections in immunocompromised hosts. There are reports of cutaneous infections, endophthalmitis, keratitis, sinusitis, neuropathy and fungemia in immunocompromised and immunocompetent adult patients. We report a 5 years old neutropenic patient with acute myeloid leukemia treated with multiple courses of chemotherapy, with a fungemia caused by Paecilomyces lilacinus (PL). His initial clinical course was characterized by fever, skin lesions, respiratory distress and shock. Blood and bone marrow cultures were positive. The patient was treated with amphotericin B and itraconazole with a good clinical response.