May-Hegglin anomaly (MHA) is a rare autosomal dominant disorder in the spectrum of myosin heavy chain-related disorders (MYH9-RD), characterized by congenital macrothrombocytopenia and white blood cell inclusions. Here, MYH9 is linked to macrothrombocytopenia and granulocyte inclusions with or without nephritis or sensorineural hearing loss.