Introduction: Onychocytic matricoma (OCM) is a benign acanthoma of the nail matrix that presents with longitudinal melanonychia and nail thickening. Only 18 previously reported cases of OCM are in the literature since it was first described in 2012. Case Presentation: The purpose of this case report was to report a unique presentation of OCM in the toenail of a Black patient as well as to review the clinical presentation, histologic features, and management of this rare entity. Previously described cases presented on the fingernails and were predominantly in white males. Conclusion: OCM is a benign entity that may mimic a nail unit melanoma or squamous cell carcinoma especially when pachyonychia is present. Despite some clinical clues to suggest a diagnosis of OCM, a nail matrix biopsy is often required to rule out malignancy.

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