The term developmental prosopagnosia refers to an impairment in the recognition of familiar faces which has been present from birth in the absence of neurological disease or birth complications. The first reported study was by McConachie (1976, Cortex, 12: 76–82) and we report here a fifteen year follow-up on this case (AB).Recently developed theoretical models postulating separate processes involved in face perception and recognition were used to guide the exploration of her functional deficit. Our investigations with AB showed that basic visuo-sensory functions (acuity, contrast sensitivity, colour, etc.) were largely intact. General face perception (e.g. distinguishing between a face and a “nonface”) was relatively well preserved. Recognition of familiar faces was severely impaired and she also showed problems with other face processing tasks (e.g. analysis of facial expression) and in object recognition. In object recognition she made errors based on visual similarity, and she had problems identifying exemplars from categories with many visually similar items. In addition, she was very poor at identifying objects or silhouettes from an unusual viewpoint. We conclude that AB has always been poor at constructing an effective internal representation sufficient to permit recognition of items which are visually difficult to discriminate. Therefore, she may not have been able to acquire useable stored representations either, because this deficit has been present since birth. This functional account was supported by subsequent studies which demonstrated a complete absence of covert face recognition.