Published rates of comorbidity between pediatric bipolar disorder (PBD) and attention-deficit/hyperactivity disorder (ADHD) have been higher than would be expected if they were independent conditions, but also dramatically different across different studies. This review examines processes that could artificially create the appearance of comorbidity or substantially bias estimates of the ADHD-BPD comorbidity rate, including: categorization of dimensional constructs, overlap among diagnostic criteria, over-splitting, developmental sequencing, and referral or surveillance biases. Evidence also suggests some mechanisms for "true" BPD-ADHD comorbidity, including shared risk factors, distinct subtypes, and weak causal relationships. Keys to differential diagnosis include focusing on episodic presentation and non-overlapping symptoms unique to mania.