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Background: Nevus spilus (NS) is a potential precursor of melanoma; the vast majority of cases reported in the literature were histologically classified as superficial spreading melanoma. Objective: To demonstrate the diagnostic value of reflectance confocal microscopy (RCM) in this subtype of congenital nevi. Methods: We report a case of a large congenital NS with equivocal clinical and dermoscopic findings in which RCM was applied for diagnosis and follow-up. Results: There was a good correlation of RCM with histopathology and a lack of dynamic changes during follow-up. Conclusion: Our observations indicate that RCM, as a non-invasive tool, can be useful for diagnosis and follow-up of clinically and dermoscopically equivocal NS.