Background:Acrodermatitis enteropathica is characterised by inflamed patches of dry red skin which then become crusted and blistered before revealing a pustulent eroded lesion. Typically these patches start near the body’s orifices before migrating to other sites, however in this patient the presentation was atypical thus delaying the diagnosis.Observations:We report a case of an atypical presentation of acrodermatitis enteropathica (AE) due to acquired zinc deficiency in a 65 year old female patient with a previous diagnosis of histologically confirmed Darier's disease. This patient's rash began on the limbs and trunk before progressing to include the perineum, oral mucosa and nose. Diffuse loss of hair on scalp, eyebrows and eyelashes was observed.Acrodermatitis enteropathica typically presents in infants, either due to an autosomal recessive genetic disorder, or after the cessation of breast feeding. In adults acquired zinc deficiency can be caused by glucagonoma syndrome, poor nutritional state, intestinal malabsorption, nephrotic syndrome and after major trauma (i.e. burns or significant surgery). In our patient low zinc levels confirmed hypozincaemia and the diagnosis of acrodermatitis enteropathica. The patient started oral zinc replacement. Within a few days her symptoms began to improve, and her hair has started to grow.Conclusion:We believe this to be an unusual presentation of acrodermatitis enteropathica due to a probable dietary zinc deficiency in a lady with pre-existing Darier's disease which may possibly have influenced the uncharacteristic clinical presentation.