A case of a fatal Scopulariopsis infection is reported in a 63-year-old lung transplantation patient with a 7-week history of dyspnea that presented initially with pericardial and pleural fluid. Because of a respiratory arrest, the patient was intubated and received positive pressure ventilation. Later, obstructive shock developed due to a pericardial tamponade requiring pericardectomy and then urgent extracorporal membrane oxygenation. The working hypothesis was of disseminated cytomegalovirus infection because cultures from bronchoalveolar lavage and transbronchial biopsy specimens were positive. The pericardial biopsy specimen showed fungal hyphae. After the patient died from the infection, the fungus was identified as Scopulariopsis acremonium. This case report describes the first case, to our knowledge, of an insidious Scopulariopsis acremonium infection in an immunocompromised lung transplant patient, underscoring the importance of a direct, invasive approach and early treatment with anti-fungal therapy in immunocompromised patients.