Primary, orbital, malignant extra-renal, non-cerebral rhabdoid tumour

Malignant rhabdoid tumour is a rare tumour outside the kidney and central nervous system. Orbital and intraocular malignant rhabdoid tumour is particularly rare with very few reports in published literature. Given the aggressiveness of this tumour and resistance to conventional chemotherapy, it is important to differentiate this tumour from other, less aggressive tumours that may have similar clinicoradiological or light microscopic features. We report one such case in a 6-week-old male child with its clinicoradiological and histopathologic features, differential diagnosis and also review the literature on the same.