Mixed gestational trophoblastic tumor is the rarest form of gestational trophoblastic neoplasia (GTN), with only 41 reported cases (Shih and Kurman. Am J Surg Pathol 22:1393–1403, 1998; Ramondetta et al. Int J Gynecol Cancer 12:312–316, 2002; Knox et al. Gynecol Oncol. 85:204–208, 2002; Shen et al. Arch Pathol Lab Med 127:e291–e293, 2003; Chen et al. Diagn Pathol 8:85, 2013; Luk and Friedlander. Case Rep Obstet Gynecol 2013:492754, 2013; Gari. BMC Res Notes 8:703, 2015; Imamura et al. Gynecol Oncol Rep 14:31–33, 2015; Zhang et al. Int J Clin Exp Pathol 8:7254–7259, 2015; Tse et al. Am J Clin Pathol 150:318–332, 2018; Kong et al. Front Oncol 9:1262, 2019; Yang et al. J Med Case Rep 14:178, 2020; Iyengar et al. BMJ Case Rep 14, 2021; Aiob et al. Int J Gynecol Pathol 41:423–430, 2022; Chatterjee et al. Am J Case Rep 23:e936451, 2022; Niu et al. Histopathology 84:325–335, 2024). Initial description of the condition reported three such cases including in the first paper characterizing epithelioid trophoblastic tumor (ETT) in 1998. In line with the general presentation of gestational trophoblastic tumors, most mixed trophoblastic tumors occur in the reproductive age (a range of 15–60 years, a median of 36 years). According to the largest series of 16 mixed trophoblastic tumors in Chinese, the interval time between antecedent pregnancy and tumor presentation is less than 12 months in 50% of the cases. Vaginal bleeding is the most common presenting symptom and notably the initial diagnosis is pure choriocarcinoma (CC) in 14/16 cases. All reported patients with mixed a gestational trophoblastic tumor have an elevated serum hCG level, which is the single, most useful biomarker for the diagnostic consideration.