BACKGROUND: Pericallosal lipomas (PCL) are rare non-neoplastic lesions, accounting for approximately 0.1% to 0.5% of all intracranial tumors and constitute 50% of all intracranial lipomas. PCL may have calcification and may continue into lateral ventricles. Neurological manifestations, including epilepsy, hemiparesis/hemiplegia, headache, and behavioral disorders, can also occur. Computed tomography (CT) and magnetic resonance image allow the diagnosis of intracranial lipomas with a high degree of reliability. CASE DESCRIPTION: A five-year-old boy was admitted to the hospital with neurological complaints. Further radiological investigation showed a pericallosal-interhemispheric lipoma associated with choroid plexus lipoma and corpus callosum dysgenesis. CONCLUSION: Pericallosal lipomas are rare mesenchymal lesions, usually asymptomatic and frequently incidental findings. However, they can progress with varying intensity headaches, signs of focal deficits, and changes in the level of consciousness. Its diagnosis can be established with CT and MRI, and usually does not require interventional methods. Surgery is reserved for symptomatic cases.