Clear-cell sarcoma is a very rare tumor, and is almost always associated with tendons or aponeuroses or is metastatic from other organs. Sporadic cases only have been reported involving primarily the bone or extending from soft tissues to surrounding bones. To our knowledge, the ilium has not been previously reported as the primary site for clear cell sarcoma.

We report a rare case of Primary clear cell sarcoma involving right ilium region in a 18-year-old boy presented with a painful swelling over right ilium and limp on right lower limb of ten month duration. He was initially suspected having tuberculosis based on clinicoradiological evaluation and diagnosis of primry clear cell sarcoma could be established on histopathology. Patient was treated with partial excision of the ilium, the remaining ilium was fused with sacrum. Stabilization was achieved with a cortical autograft harvested from the right fibula and fixation with a titanium plate.

The patient had no local recurrences but the plate holding ilium to sacrum broke and was removed in the subsequent surgery after which he developed Trendelenberg’s gait.