Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Here we describe a girl of 4½ years age admitted with the complaints of, low back pain for 2 months, gradually worsening weakness in both lower limbs for the same duration along with urinary incontinence and constipation for 1 month. On examination she was afebrile, mildly pale, no lymphadenopathy, having normal vital signs and anthropometrically well thriving. Back and spine examination showed angulation of spine involving T₁₁ - L₄, without paravertebral swelling. Neurological examination of lower limbs revealed decreased muscle tone, diminished muscle power (3/5), both knee and ankle jerks were also diminished and bilaterally equivocal planter response, sensory function of all modalities were impaired. MRI findings were suggestive of ependymoma. Consultation was done with department of neurosurgery and they did the relevant surgery. Tissue histopoathology and immune histochemistry confirmed the diagnosis of primitive neuroectodermal tumor. Finally the child was referred to Paediatric Haemato-oncolgy department for further management.