The SLC29A3 gene has recently been found mutated in rare patients with inherited syndromic histiocytosis resulting in 4 overlapping phenotypes including H syndrome, pigmented hypertrichosis with insulin-dependent diabetes mellitus (PHID), Faisalabad histiocytosis (FHC) and sinus histiocytosis with massive lymphadenopathy (SHML) or Familial Rosai–Dorfman disease (RDD) [3]. This evidence concerns the gene SLC29A3 and sinus histiocytosis with massive lymphadenopathy.