Uncommon Microascus cirrosus (M. cirrosus) species have been reported to cause an increasing number of subcutaneous and invasive fungal infections worldwide; since the first human infection was reported in 1992, seven cases have been reported in PubMed. The present study reports a novel genotype named M. cirrosus SZ 2021 isolated from a patient undergoing hematopoietic stem cell transplantation, who exhibited extensive drug resistance and suffered a fatal pulmonary infection. This isolated M. cirrosus was cultured and determined by morphological observation, multi-locus sequence typing, matrix-assisted laser desorption and ionization time-of-flight mass spectrometry, and whole genome sequencing by next-generation sequencing. The whole nucleotide sequence (32.61 Mb) has been uploaded in the NCBI database (PRJNA835605). In addition, M. cirrosus SZ 2021 was not sensitive to the commonly used antifungal drugs, including fluconazole, amphotericin B, 5-flucytosine and caspofungin. The current literature on human infections by M. cirrosus was reviewed to closely define the comprehensive clinical characteristics and etiological identification. In brief, the present study identified a new M. cirrosus and summarized the clinical characteristics of fungal pneumonia by M. cirrosus species. Complete laboratory identification methods from morphology to gene sequencing were also established for an improved etiological identification and further investigation into the real prevalence of invasive pneumonia by M. cirrosus.