Background: Enchordoma of the distal phalange of the thumb is extremely rare. Case presentation: We report a case of 31-year-old man who presented with a pathological fracture of the left thumb. Imaging evaluation revealed a lytic lesion and surgical curettage with bone graft was performed after fracture healing. Histological examination confirmed the diagnosis of enchordoma. The postoperative period was uncomplicated without signs of recurrence. Conclusion:Lytic lesions in the thumb are uncommon occurrences and necessitate a comprehensive examination to determine their potential causes. Given the significant functional role of the thumb compared to other fingers, it is crucial to undergo radiological assessment and further investigation of these lytic lesions.