BACKGROUND Pseudoainhum is a rare disease characterised by gradual fibrous constriction of the fingers and/or toes eventually resulting in their amputation. In this article, we report the first case seen in Morocco, highly unusual in terms of its severity. CASE REPORT A 46-year-old woman with no toxic habits was hospitalised for spontaneous amputation of the fingers and toes. This condition began when the patient was 12 years old with the appearance of a circular constriction band at the base of the fifth toe, eventually resulting in its loss. The patient gradually lost all her other toes and fingers except for the first joint of her left index finger. There was no family history of any similar condition. Clinical examination also revealed perforating plantar disease in two of the stumps and peripheral neuropathy in all four limbs, comprising predominantly axonal disease responsive to electromyogram. Amputation of the index finger was completed and histological examination of the removed section showed nothing unusual, with no signs of diabetes. DISCUSSION The peculiarity of our case resides in the exceptional severity of these amputations and their association with isolated polyneuropathy. Pseudoainhum has been described chiefly in patients with congenital keratoderma, certain systemic diseases, diabetes and alcohol dependence. A number of etiopathogenic hypotheses have been suggested: traumatic, infectious, vascular, neurological and genetic processes.