Introduction: Osteopoikilosis and enchondroma existing together have not been reported in literature, and this is the first report of the management of the same.
Case report: A 26-year-old male presented with dull aching pain with swelling around the knuckle of the left index finger of 1 month duration. On examination, there was a swelling of approximately 1x1 cm on the dorsal aspect. Typical radiographic changes of osteopoikilosis and enchondroma were present.
Conclusion: Enchondroma coexistence with osteopoikilosis is rare. Diagnosis is suspected on plain radiographs and confirmed by the histopathologic study. Enucleation of the tumor with bone graft provides good results.
Keywords: Osteopoikilosis; bone graft; enchondroma; hand; tumor.