Purpose: To report a case of primary hepatoid adenocarcinoma of the orbit.
Observations: An adult patient was referred for evaluation of an orbital mass. Histopathology of the orbital biopsy indicated a carcinoma with hepatoid features. Laboratory studies revealed normal liver function tests, elevated serum alpha-fetoprotein, and whole-body positron emission tomography/computed tomography scan showed no evidence of liver involvement or an alternative primary origin.
Conclusions and importance: To the authors' knowledge, this is the first reported case of primary hepatoid adenocarcinoma of the orbit.
Keywords: Alpha-fetoprotein; Hepatoid adenocarcinoma; Orbital tumor.