Pseudoainhum is an affection characterized by the appearance of a constricting band around a digit or limb which may lead to spontaneous amputation. There are various etiologies which can be either congenital or resulting from a concomitant disease. We report herein an original case which occurred during lamellar ichthyosis.We present the case of a thirty-year-old woman affected by a severe form of lamellar ichthyosis who, in a few months, developed a pseudoainhum of the third left finger. Biological results were normal. X-rays of this finger showed distal resorption of the bone. The painful evolution and the absence of any conservative treatment compelled us to propose amputation of the third phalanx.Other cases of pseudoainhum have been observed in many disease responsible for vascular or neurological abnormalities. They can also result in physical traumatisms or be associated with many dermatoses, such as hereditary palmar and plantar keratodermas. As far as we know, this lesion has never been described during lamellar ichthyosis but could result in the keratinization disorders observed in this congenital ichthyosis.