Dupuytren’s disease (DD) typically affects the palmar fascia and proximal digital structures, with distal interphalangeal joint (DIPJ) involvement considered rare. True extension of DD into the volar pulp has not been previously documented. Distal lesions may be misdiagnosed as neoplastic or inflammatory masses, and optimal management of isolated distal cords remains uncertain. We present the first histologically confirmed case of DD extending beyond the DIPJ into the volar pulp, accompanied by a systematic re-view of reported DIPJ-dominant DD. A 30-year-old right-hand-dominant male presented with a two-year history of progressive flexion deformity of the little finger. Examination demonstrated a 90° proximal inter-phalangeal joint and 55° DIPJ contracture. Ultrasound and MRI showed a well-circumscribed soft-tissue lesion along the radial middle phalanx but did not suggest DD. Open exploration via an ulnar digital approach revealed a discrete DD cord ex-tending distally beyond the DIPJ into the volar pulp, closely associated with the ulnar neurovascular bundle. Limited fasciectomy achieved full correction without neurovas-cular compromise. Histopathology confirmed classic DD. At the twelve-month follow-up, the patient maintained full extension and function with no recurrence. This study reports the first confirmed case of DD extending into the volar pulp and highlights that atypical distal DD can occur even in young patients. Imaging may fail to identify DD in uncommon sites, reinforcing the importance of clinical suspicion. Limited fasciectomy remains safe and effective in the distal phalanx. Recognition of this phenotype may improve diagnostic accuracy and guide tailored operative planning.