Desmoplastic infantile astrocytoma (DIA) is a rare, supratentorial, dural-based, large cystic tumor that usually arises in the first 24 months of life. However, non-infantile cases were also reported in the literature. Desmoplastic infantile astrocytoma and desmoplastic infantile ganglioglioma (DIG) are both classified as grade I astrocytoma by the World Health Organization (WHO). Grossly, DIA/DIG are large tumors composed of solid and cystic portions. Although large in nature, they are slow-growing tumors, with good prognosis after complete surgical removal, and rarely require a chemotherapy or radiotherapy. However, there have been few cases of DIA that demonstrated malignant features and/or spontaneous recurrence or metastases which necessitates close-up monitoring after surgical intervention. Herein, we report a case of an 18-month-old boy who presented with progressive head enlargement that was discovered to be due to a large left frontal predominantly cystic tumor. The patient underwent subtotal resection (STR) and was diagnosed as DIA on histopathological examination. Over a duration of 18 months of follow-up, the patient's status deteriorated, and he eventually died. .