To test the hypothesis that the high rate of genomic alterations found in our BI-ALCL sample could be related to mutations in genes involved in DNA damage repair and cell cycle control, we performed a targeted NGS with 26 cancer susceptibility genes, which revealed a germline variant of uncertain significance V1027L of the mismatch repair gene MSH6 (allele variant 49.80%). The gene discussed is MSH6; the disease is anaplastic large cell lymphoma.