Abstract
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, locally aggressive cutaneous sarcoma. DFSP in the periocular region is exceedingly rare, leading to diagnostic and surgical challenges due to anatomical constraints in the periocular region. Precise diagnosis is essential to guide appropriate surgical management and prevent recurrence. CASE SUMMARY: A 32-year-old female presented with a recurrent tumor in the medial canthus, previously diagnosed as a solitary fibrous tumor in an outside institution. After complete radiological and systemic workup, she was scheduled for a wide local excision followed by reconstruction after getting tumor clear margins on frozen section. Histopathology confirmed DFSP, characterized by storiform spindle cell proliferation, diffuse cluster of differentiation 34 positivity, and signal transducer and activator of transcription 6 negativity. CONCLUSION: This case highlights the challenges in the diagnostic and surgical management of DFSP in periocular tumors. Comprehensive surgical excision with appropriate reconstruction is critical for achieving oncological control while preserving aesthetics and function.