Bipolar I disorder occurs in approximately 1% of the adult population, and it affects women and men equally. Women develop bipolar II disorder, bipolar depression, mixed mania, and a rapid-cycling course of illness more commonly than men and are at greater risk of such comorbid conditions as alcohol use problems, thyroid disease, medication-induced obesity, and migraine headaches. The treatment of bipolar disorder remains challenging. Although lithium reduces symptoms and prevents recurrence with good efficacy, a significant number of patients stop taking it. Furthermore, several anticonvulsants and antidepressants are prescribed off label for acute episodes and prophylaxis despite the lack of adequate research support. Psychotherapy may alleviate mania or depression and improve treatment compliance, yet its ability to prevent relapse remains uncertain. Changes throughout the reproductive cycle also have an impact on the onset and presentation of bipolar symptoms and the choice of treatment. This article provides an overview of common presentations and comorbidities, along with approaches to evaluation and treatment of women with bipolar disorder.