Steatocystoma is considered as forme fruste of pachyonychia congenita II and has been reported to be associated with mutations of keratin 17 gene in familial disease cases.[8‐10] Steatocystoma has been found in association with various conditions like multiple keratoacanthomas, rheumatoid arthritis, hyperkeratotic lichen planus, hidradenitis suppurativa, acrokeratosis verruciformis of Hopf, ichthyosis, koilonychias, and bilateral preauricular sinuses.[11, 12]. The gene discussed is KRT17; the disease is familial keratoacanthoma.