The loss of hENT3 has been associated with clinical spectrum disorders of SLC29A3 gene defects, also known as histiocytosis-lymphadenopathy plus syndrome comprising features of 4 histiocytic disorders sharing overlapping clinical manifestations previously thought to be distinct: Faisalabad histiocytosis (FHC), sinus histiocytosis with massive lymphadenopathy (SHML) or Familial Rosai–Dorfman disease (RDD), H syndrome, and pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID) [3, 5, 6]. Here, SLC29A3 is linked to H syndrome.