Urticaria is one of the most common skin diseases that can basically be diagnosed with visual inspection, but by this reason, one may fall into a pitfall while he is diagnosing and treating a patient as usual. In the Japanese Guidelines for Diagnosis and Treatment of Urticaria 2018, hereditary angioedema, urticaria pigmentosa and cryopyrin-associated periodic fever syndrome have been picked up as urticaria-related hereditary diseases. Furthermore, novel genetic mutations have recently been identified in families with hereditary angioedema with normal activity of C1 inhibitor. In addition, NLRP12-related periodic fever syndrome, PLCG2-related antibody deficiency and immune dysregulation, and NLRC4 abnormalities have been reported as familial cold autoinflammatory syndrome-like diseases. More recently, the causative gene for familial vibratory urticaria/angioedema has also been identified. By deepening knowledge of these hereditary diseases that should be distinguished from urticaria, one can not only make a proper diagnosis of rare diseases in the early stage to prevent serious attacks and complications, but also understand more deeply and widely the pathomechanisms of urticaria and angioedema.