Previous studies have suggested that typically developing 6-month-old infants are able to discriminate between small and large numerosities. However, discrimination between small numerosities in young infants is only possible when variables continuous with number (e.g. area or circumference) are confounded. In contrast, large number discrimination is successful even when variables continuous with number are systematically controlled for. These findings suggest the existence of different systems underlying small and large number processing in infancy. How do these develop in atypical syndromes? Williams syndrome (WS) is a rare neurocognitive developmental disorder in which numerical cognition has been found to be impaired in older children and adults. Do impairments of number processing have their origins in infancy? Here this question is investigated by testing the small and large number discrimination abilities of infants and toddlers with WS. While infants with WS were able to discriminate between 2 and 3 elements when total area was confounded with numerosity, the same infants did not discriminate between 8 and 16 elements, when number was not confounded with continuous variables. These findings suggest that a system for tracking the features of small numbers of object (object-file representation) may be functional in WS, while large number discrimination is impaired from an early age onwards. Finally, we argue that individual differences in large number processing in infancy are more likely than small number processing to be predictive of later development of numerical cognition.