We report a rare case of systemic lymphadenitis and hepatic involvement due to Exophiala (Wangiella) dermatitidis in a pediatric patient. An 8-year-old immunocompetent boy with chronic fever was examined through the use of sonography and CT scan which demonstrated cervical and mesenteric lymph node enlargement and numerous small hepatic lesions. The etiologic agent was isolated by means of lymph node aspiration. The fungus was identified by its morphological characteristics and through DNA sequencing of the internal transcribed spacer region of rDNA. Despite initial amphotericin B and voriconazole therapy, the child's jaundice subsided and he died 7 months later. In addition to pathogenic aspects of Exophiala dermatitidis, the diagnostic approaches and relevant therapeutic strategies are discussed.