In this report, we describe an isolated concentric hypertrophic cardiomyopathy in a 3-year-old proband carrying compound heterozygous variants in TSFM, the c.997C>T [p.(Arg333Trp)] variant and the c.355G>C [p.(Val119Leu)] variant, both in highly conserved regions of the TSFM protein. The gene discussed is TSFM; the disease is hypertrophic cardiomyopathy.