Here we present the case of a 23 year old Caucasian patient with recurrent undifferentiated sarcoma, where tumor profiling reported pathogenic alterations in BRCA2 and MLH1. Constitutional analysis confirmed a pathogenic BRCA2 alteration and led to reclassification of the MLH1 alteration as a variant of uncertain significance. The gene discussed is MLH1; the disease is undifferentiated (embryonal) sarcoma.