Acquired ungual fibrokeratomas or AUFKs are benign recurrent fibrokeratotic tumors of the nail unit of uncertain etiology. Little is known about the optimal modality to treat these lesions successfully. No systematic review addressing ungual fibrokeratomas has been published to date. This study aimed to summarize all the published data regarding the diagnostic and therapeutic challenges of AUFKs. On August 9, 2019, the researchers and doctors conducted a systematic search of Medline and Cochrane databases. All the studies describing the treatment of ungual fibrokeratomas, procedure description, pathological findings, outcome, and follow‐up period were included in this review.

After a full‐text article review, it included 103 articles, representing a total of 78 lesions. The commonly used fibrokeratomas treatments were complete tumor resection, i.e., 90.5%, shave excision, i.e., 8.3%, and cryotherapy, i.e., 1.2%. Complete surgical excision yielded the highest cure rate among all treatment modalities (90.8%) compared to partial surgical excision (28.6%) and cryotherapy (0%). The mean follow‐up period was 12.1 months (1–144). Complete surgical removal preserving the matrix showed the highest cure rates and should be considered first‐line treatment for Acquired ungual fibrokeratomas. Future adequately designed randomized control trials are warranted to compare different treatment modalities.