Background <p>Pilocytic astrocytoma (PA) is the most commonly diagnosed primary brain tumor in children. The majority of documented astrocytic tumors are low-grade, with PA being the most prevalent. Pilomyxoid astrocytoma (PMA), a variant of PA, generally exhibits a more aggressive behavior compared to other variants. Due to its rarity and overlapping features with other entities in the literature, this case has been reported and discussed.</p> Case presentation <p>This report describes a case involving a 2-year-old boy presenting with a well-defined, lobulated lesion in the suprasellar region, alongside a review of relevant literature. During squash cytology from the frozen section, a low-grade tumor was identified, composed of cells with elongated, fibrillary cytoplasm and round to oval nuclei with dispersed chromatin. Histological examination confirmed the diagnosis, and we will provide a detailed account of the lesion’s histological and radiological features. Additionally, the report discusses the treatment options and the patient’s outcome.</p> Conclusion <p>The diagnosis is challenging due to histological similarities to classic pilocytic astrocytoma, a difficulty often exacerbated by the limited tissue obtained from small biopsies in unresectable tumors. Squash cytology can only be used as a screening tool. Although classified as low-grade pediatric gliomas, PMA tends to be more aggressive than PA. Surgical resection remains the preferred treatment for effective disease management, although there is a risk of recurrence.</p>