A 27-year-old male presented with headache, vomiting and blurring of vision for 1 wk duration. The patient’s headache was of gradual onset, throbbing in nature, worse in the morning with associated projectile vomiting and blurring of vision. The headache was relieved temporarily by analgesia but progressively worsened over the course of a week. The patient had history of acute lymphoblastic leukaemia at the age of four and was treated according to the Berlin-Frankfurt-Munster Protocol. Neurological examination on the day of admission revealed bilateral reduction in visual acuity. Visual acuity for the right eye was light perception and for the left eye was 6/6. No other neurological deficit was noted. Systemic examinations were unremarkable. Blood investigation findings were normal. Magnetic resonance imaging of the brain revealed a heterogeneously enhanced hypothalamic tumour. The lesion measured 2 cm × 1.5 cm, located mainly at the hypothalamus with extension into the optic chiasm. No pituitary stalk and pituitary gland involvement was detected. Stereotactic biopsy of the tumour was performed. Histopathological examination of the specimen showed focus of tumour infiltration that displayed rhabdoid morphology. Nuclei were eccentrically placed, hyperchromatic and pleomorphic with coarse chromatin. There was an ample amount of eosinophilic cytoplasm. Mitotic figures were seen. The cells stained positive for vimentin, cytokeratin (CK) AE1/AE3, glial fibrillary acidic protein (GFAP), cluster of differentiation (CD) 68 and CD138. Synaptophysin, human melanoma black 45, CK7, CK20, desmin, smooth muscle actin, CD79, CD30, terminal deoxynucleotidyl transferase, placental alkaline phosphatase, and leucocyte common antigen were negative upon staining. Ki67 was more than 20%.