Naxos disease is a rare cardiocutaneous syndrome with autosomal recessive inheritance (Orphanet 2024). It was first described in 1986 (Protonotarios et al. 1986). He reported nine cases from four families on the Greek island of Naxos. Patients had dense, rough, and bristly scalp hair, as well as structural and electrical heart disorders, including right ventricular enlargement, EKG abnormalities, episodes of ventricular tachycardia, and sudden death in one patient (Protonotarios et al. 1986). Studies show that heart disease is the same in both Naxos disease and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) except that Naxos disease is autosomal recessive, while most hereditary dilated cardiomyopathies are autosomal dominant (McKoy et al. 2000; Islam et al. 2017). However, according to the WHO classification, Naxos disease has been considered the recessive subtype of ARVC/D since 1995 (Richardson 1996). Woolly hair is present at birth, while palmoplantar keratoderma develops during the first year of life when the infants begin to use their hands and feet (Protonotarios and Tsatsopoulou 2006). Cardiomyopathy appears clinically in adolescence and shows 100% penetration (Protonotarios and Tsatsopoulou 2006). Arrhythmias usually present as syncope or sustained ventricular tachycardia of the right bundle branch block, but sudden death may also be the first manifestation of the disease (Islam et al. 2017). The prevalence of the Naxos disease in the Greek islands might be around 1:1000, but it has also been reported in other regions such as India, Bangladesh, Turkey, Israel, and Saudi Arabia (Protonotarios and Tsatsopoulou 2006). All the clinical manifestations are shown in Table 1 (Online Mendelian Inheritance in Man O 2024).