Altogether, GTLS represents about 17% of families with inherited thrombocytopenia with an identified genetic defect being followed up in our hospital, being more frequent than TUBB1 (13%), ACTN1 (10%), ANKRD26 (11%), and MYH9 (10%) related thrombocytopenia, and biallelic (7%) or monoallelic (6%) Bernard Soulier Syndrome. The gene discussed is MYH9; the disease is Thrombocytopenia.