Hereditary palmoplantar keratodermas (PPKs) consist of a heterogeneous group of hyperkeratotic disorders involving mainly the palms and the soles. Nagashima-type palmoplantar keratosis (NPPK, MIM615598) is an autosomal recessive diffuse non-epidermolytic palmoplantar keratosis caused by mutations in SERPINB which is abundantly expressed in the skin of the whole body, especially in the stratum granulosum, indicating its role in epidermal homeostasis. An 18-year-old male patient visited our department with erythematous thick skin on the palms and soles since childhood. On physical examination, diffusely distributed, mild and nonprogressive palmoplantar erythema and hyperkeratosis were seen beyond the palmar and plantar skin. Histologic examination of the skin biopsy revealed hyperkeratosis, parakeratosis, hypergranulosis and orthokeratosis. We carried out sequencing of the SERPINB7 via using Sanger and wholeexome sequencing. We identified one heterozygous recurrent mutation in SERPINB7. We report herein the first case of NPPK in Korea