We present an unique case of pachyonychia congenita associated with steatocystoma multiplex. A 33-year-old Japanese man had thickening and gray-brown dicoloration of all nails and a large number of nodules or tumors over his entire skin. No palmar and plantar hyperkeratosis, leukokeratosis of the mucous membranes, or follicular keratosis were observed. Histology of these tumors revealed the typical features of steatocystoma multiplex.