Case reportPitfalls and uncertain prognosis in pathological diagnosis of psammomatous melanotic schwannoma

•Woman aged 20 presented with a pelvic mass with atypical histological features.•Mass was identified as a psammomatous melanotic schwannoma.•Patient was later found to have family history of Carney's complex.•Biomarkers and morphological distinctions are crucial for differential diagnosis.

A 20-year-old woman presenting with a pelvic mass identified as a psammomatous melanotic schwannoma (PMS) with atypical histological features was later found to have family history of cardiac myxomas consistent with Carney's complex. The BRAF V600E mutation was absent in the tumor.