Human Lasiodiplodia theobromae infection has not been reported frequently. We report the first case of invasive L. theobromae nasal and neck infection. A 66-year-old male visited our hospital with anemia and general weakness. He showed pancytopenia, and his bone marrow examination revealed markedly decreased hematopoietic cells. The patient was presumed to have iatrogenic aplastic anemia due to mushroom toxicity. He began treatment for multiple organ infections with broad-spectrum antibiotics and antifungal agents. During hospitalization, he complained of nasal obstruction and left neck lymph node enlargement. A mass-like lesion was observed, and a nasal mass biopsy was performed. The mass was identified as a fungal ball. He underwent surgical excision for the nasal mass and the neck lymph node. The pathologic examination indicated an invasive fungal infection, and the lymph node revealed chronic granulomatous inflammation with fungal infection. 18s rRNA sequencing revealed that the sequence shared 99 % identity with L. theobromae. The nasal mass fungus was identified by internal transcribed spacer region sequencing from pathologic paraffin sections. The obtained sequence corresponded to Lasiodiplodia or Macrophoma. The sequence corresponded to the neck discharge sequence results. Hence, the patient was diagnosed with invasive fungal sinusitis with neck lymph node involvement caused by L. theobromae. To our knowledge, this is the first report of L. theobromae infection in Korea and the first report of invasive L. theobromae fungal sinusitis in the literature. We should include more precise evaluations of additional novel fungal species as possible candidates.