Primary adrenal teratoma is a rare type of cancer. Of the 338 patients who underwent adrenalectomy during this study, only 2 (aged 69 and 29 years) were diagnosed with adrenal teratoma and underwent laparoscopic retroperitoneal adrenalectomy. For the purposes of the present study, the term 'adrenal teratoma' was searched in the PubMed database, and 237 articles published between June 1952 and March 2017 were retrieved. However, we were only able to identify 10 relevant studies. In total, these studies reported a series of 18 cases of primary adrenal teratoma in patients aged >16 years, another 8 cases of adult adrenal retroperitoneal teratoma, and 7 cases of adrenal teratoma in children aged <16 years. In the 18 cases aged >16 years, the age range was 17-61 years (mean ± standard deviation, 33.06±15.47 years), the median tumor diameter was 8.25 cm and 13 patients (72.22%) were female. Almost all patients underwent laparoscopic surgery between 2006 and 2017 (75%). Among the 7 cases of adrenal teratoma in children under the age of 16 years, 5 cases (71.43%) were male, the median tumor diameter was 10 cm, the oldest patient was aged 8 years, 5 cases (71.43%) were selected for open surgical resection of the tumor, and 5 cases (71.43%) were followed up without recurrence or death. These data indicate that primary adrenal teratomas in children are rarer compared with adults. Although the data are limited, it was observed that the clinical symptoms of primary adrenal teratoma are not typical, the preferred treatment is retroperitoneal laparoscopic surgery, and the prognosis is favorable. The aim of the present study was to elucidate the clinical characteristics associated with primary adrenal teratoma, in order to further raise awareness of this rare disease.