Objective: The objective of this article is to analyze the maternal and fetal outcomes of pregnancies that present with arteriovenous malformations (AVMs). Methods: A literature review was performed that analyzed 65 cases of AVM during pregnancy previously reported in English literature. Results: Sixty-five cases of pregnancy-associated AVM were identified. The patients' ages ranged from 16 to 45 years, with a mean of 28 +/- 4.9 years. Sixteen cases (24.6%) were pre-existing AVMs. There were 54 cases (83.1%) of AVM ruptured during pregnancy and postpartum: Six cases (11.1%) were in the first trimester, 24 (44.4%) were in the second, 22 (40.7%) were in the third trimester and two (3.7%) were postpartum. Unfavorable maternal clinical outcome (modified Rankin Scale (mRS) >= 2) was identified in 20 cases (30.8%) and abortion occurred in 10 cases (15.4%). There were three maternal deaths, yielding a case mortality rate of 4.6%. Fifty-three fetuses were born via cesarean section in 42 cases and vaginal delivery in 10 cases; 48 were in good health, three were temporarily intubated, one was macrosomic and one died. In univariate analysis, AVM hemorrhage presentation was significantly associated with a poor maternal outcome (mRS >= 2) (p = 0.030); however, not significantly associated with fetus risk (p = 0.864). Gestational age was not significantly associated with poor maternal outcome (p = 0.875) or fetal risk (p = 0.790). Conclusion: AVM hemorrhage presentation was significantly associated with poor maternal outcome. Pre-existing ruptured AVM may not be associated with fetal risk. Gestational age of AVM rupture was not significantly associated with poor maternal outcome or fetal risk.