Objective: Synovial sarcoma usually presents with spindle cell morphology with or without epithelial differentiation. Extensive rhabdoid differentiation is a very rare feature with only few cases described in literature.
Case Report: We present two cases of synovial sarcoma with rhabdoid differentiation along with their clinical follow-up. Both cases had tumor in the vicinity of joints and showed lung metastasis during follow-up inspite of R0 resection.
Conclusion: We emphasised that extensive rhabdoid differentiation can be deceptive and challenging for diagnosis in small biopsies and also show an aggressive clinical course with dismal prognosis. Awareness of this rarely described unusual and aggressive histomorphological subtype is prudent due to its distinct diagnostic, prognostic and therapeutic implications.