Several mutations have been identified so far in the Italian population, all of them appearing to cause a loss of function [15], [18]–[26].Starting from 2004 we have investigated 87 consecutive index cases, with the presence of multiple angiomas and/or with a positive family history (FCCM); we identified mutations in over 97.7% of FCCM cases Among the positive cases, KRIT1/CCM1 accounts for 68.9%; MGC4607/CCM2 for 18.0% and PDCD10/CCM3 for 13.1%. The gene discussed is KRIT1; the disease is hemangioma.