By applying WES analysis in a family affected by HS we found a rare frameshift insertion in exon 4 of DCD gene in heterozygosis (rs538180888; g.54645236_54645237insT; NM_053283.4:c.225dup; NP_444513.1:p.Ala76Fs) in both HS cases (Patient 1 and Patient 2) and in the 11-year-old daughter of Patient 1, while the identified variant was not detected in the mother of Patient’s 1 daughter, healthy control who reported not to be affected by any known disease. Here, DCD is linked to histiocytic sarcoma.