To our knowledge, this is the first time that the NOD2 2722G > C (rs2066845) variant substituting a Glycine to Arginine at codon 908 in exon 8 in the leucine rich repeat (LRR) domain of the NOD2 protein is reported in a case of familial sarcoidosis with late and typical presentation of the disease. This evidence concerns the gene NOD2 and sarcoidosis.