Objectives: (1) Describe a presentation of first branchial cleft anomalies. (2) Compare outcomes of first branchial cleft to other branchial cleft anomalies with attention to otologic findings. Methods: For this case-controlled study, databases at Seattle Childrens Hospital were queried by International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes for pediatric branchial cleft cases from 2004 to 2013. Inferential analysis was performed using unpaired t test. Measurements of risk were calculated using Fisher’s exact test. Results: The query identified 104 subjects; 24 (23.1%) of whom had first branchial cleft anomalies, the remaining 80 (76.9%) had second or third branchial cleft anomalies. First branchial cleft anomalies were diagnosed at an older age, 2.65 years (SD = 3.3) versus 1.66 years (SD = 4), P value .28 (not significant). They also presented with a range of otologic findings, including otorrhea (25%), otitis media (29.2%), tympanic membrane web (26.1%), and cholesteatoma (16.7%). They had greater risk of requiring primary incision and drainage: 14 (58.3%) versus 3 (3.8%), odds ratio (OR) 15.12, 95% confidence interval (CI) [3.8, 88.9], P value <.0001. They were also more likely to have recurrent disease: 7 (29.1%) versus 3 (3.8%), OR 7.6, 95% CI [1.59, 49.1], P value .008. They were more likely to undergo additional surgery: 6 (26.1%) versus 4 (5%), OR 4.91, 95% CI [1.07, 25.72], P value .04, often related to residual ear disease. Conclusions: Children with first branchial cleft anomalies present with a range of otologic manifestations that increase the risk of persistent disease and that may require specific treatment such as tympanoplasty.