MONA syndrome has multiple differential diagnoses, including Ter Haar syndrome (homozygous mutation in the TKS4 gene) [11], multicentric carpotarsal osteolysis syndrome [12], Winchester syndrome [13], juvenile idiopathic arthritis, and mucopolysaccharidosis type I as Scheie syndrome [14]. Here, SH3PXD2B is linked to multicentric osteolysis-nodulosis-arthropathy spectrum.