A 6‐year‐old boy with left temporal lobe mass

or fusions are frequently observed in DIA/DIG in absence of CDKN2A/B homozygous deletion, with BRAF V600E mutation frequently reported particularly in non-infantile cases of DIA/DIG, as seen in this case. 2 This case aims to present a DIA with atypical features, posing a diagnostic challenge due to its close resemblance to meningioma both in imaging and macroscopic appearance. On imaging, the tumor was relatively limited in size and lacked the typical giant cystic component seen in most DIA/DIG. 3 Additionally, rigid peripheral calcifications resembling those associated with meningioma were observed, which are not characteristic of DIA/DIG. Intraoperatively, the tumor was primarily parenchymal in location; however, portions of its calcified components had breached the lep-tomeninges and exhibited focal adhesions to the dura mater. In addition, the presence of sandy-like tissue surrounding it macroscopically also contributed to the initial diagnostic confusion. In intraoperative frozen sections, spindle-shaped tumor cells can be observed in both meningiomas and DIA/DIG. A thorough analysis of postoperative immunohistochemical staining confirmed the diagnosis of DIG, characterized by the presence of glial markers OLIG2 and GFAP, along with scattered synaptophysin-positive neurons. We hypothesize that the atypical features observed in this case may be partially attributed to the tumor ’ s special location in the middle cranial fossa, where spatial constraints and compression may have influenced its growth pattern. Given the favorable prognosis associated with complete surgical resection, no additional treatment was planned for this patient. The follow-up strategy includes routine evaluations with periodic imaging and clinical assessments to monitor for any signs of recurrence or complications.