Background: Fibro-osseous pseudotumor of the digit (FOPD) is a rare benign ossifying lesion of the soft tissues of fingers or toes. It may mimic other periungual tumors or malignant lesions, leading to diagnostic confusion. Case presentation: We report a case of a 46-year-old woman with a 6-month history of a painless, progressively enlarging nodule of the right index finger. The lesion was excised for cosmetic concern. Histopathological examination revealed a well-circumscribed spindle-cell proliferation with osteoid and trabecular bone formation, overlaid by hyperplastic and orthokeratotic epidermis. No cytologic atypia or mitotic activity was noted. Conclusion: Recognition of FOPD is essential to avoid misdiagnosis and overtreatment. Molecular studies may show USP6 rearrangements, supporting its classification as a benign transient neoplasm. Complete local excision is curative with excellent prognosis.