Furthermore, in two cases of HLRCC with Programmed Cell Death-Ligand 1 (PD-L1) positivity, the efficacy of immunotherapy was also reported [13, 14].Here, we describe a case of aggressive HLRCC in a 33-year-old female who exhibited a novel heterozygous germline insertion mutation in exon 8 of the FH gene (c.1126 C > T; p.Q376*) and had positivity of PD-L1. Here, FH is linked to hereditary leiomyomatosis and renal cell cancer.