Acquired ungual fibrokeratomas (AUFKs) are benign recurrent fibrokeratotic tumors of the nail unit of uncertain etiology. Little is known about the optimal modality to successfully treat these lesions. No systematic review addressing ungual fibrokeratomas has been published to date.The aim of this study is to summarize all the published data regarding diagnostic and therapeutic challenges of AUFKs.On August 9, 2019, a systematic search of Medline and Cochrane databases was conducted. All the studies describing the treatment of ungual fibrokeratomas, procedure description, pathological findings, outcome, and follow-up period were included in this review.After full-text article review, 103 articles were included, representing a total of 78 lesions. The commonly used treatments for fibrokeratomas were complete tumor resection (90.5%), shave excision (8.3%), and cryotherapy (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 as first-line treatment for AUFKs. Future adequately designed randomized control trials are warranted to compare different treatment modalities.