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Background. Distal digital keratoacanthoma (DKA) is an uncommon tumor difficult to diagnose clinically, and even histologically, with certainty.

Objective. Our purpose is to report on two new cases and to discuss the clinical, histologic, and differential diagnosis.

Methods. We have reviewed all well documented cases published in the literature.

Results. No single diagnostic criterion is sufficiently sensitive and specific to be pathognomonic.

Conclusion. The diagnosis of DKA should be based on the correlation of clinical, radiological and pathologic findings, but the tumor is frequently diagnosed histologically as “squamous cell carcinoma, keratoacanthoma type” or as keratocarcinoma.