Retinoblastoma very rarely presents as vitreous haemorrhage. We are presenting a case of atypical presentation of retinoblastoma in a 3-year-old girl. She initially came with right eye vitreous haemorrhage of unknown cause. B-mode ultrasound showed dense vitreous opacity without evidence of mass. Initial MRI Brain/Orbit was inconclusive. Diagnostic vitrectomy was performed and noted thickened abnormal retina which was suspicious for retinoblastoma. The parents refused for enucleation for diagnostic histopathological examination and opted for conservative management. Repeated MRI Brain/Orbit done six months later showed disease progression through optic nerve involvement and suggestive of retinoblastoma. The parents were re-counselled for enucleation however refused and defaulted. 2 months later, the child was brought back with proptosed and disorganized eye. This time, they agreed for intervention. The patient underwent three cycles of chemoreduction therapy before enucleation. After enucleation, she received six cycles of adjuvant chemotherapy. She was well with no disease recurrence at two-year post treatment.