Pachydermodactyly (PDD) is a rare, benign condition characterized by swelling and thickening of the periarticular skin, most commonly at the proximal interphalangeal joints. Diagnosis is routinely made through correlation of clinical, histopathologic, and radiographic findings. Here, we report a case of PDD in a 25-year-old male, with emphasis on the clinical and histopathologic differential diagnosis and potential diagnostic pitfalls.