Large solitary café au lait spots: a report of 5 cases and review of the literature

The presence of multiple café au lait spots (CALSs) has been well described and associated with several neurocutaneous and genetic syndromes including, most commonly, neurofibromatosis. However, scant literature exists regarding the clinical significance of the large solitary CALS. We describe 5 patients with congenital large solitary CALSs without associated abnormalities. Our cases and review of the literature suggest that large solitary CALSs are uncommon benign lesions that most likely are not associated with any neurocutaneous syndromes or developmental anomalies.