Primary ciliary dyskinesia is a genetically heterogeneous motile ciliopathy characterized chronic respiratory disease, laterality defects, hydrocephalus, and infertility, caused by impaired function of motile cilia. has recently emerged as a novel PCD candidate gene, but its role in vertebrate cilia remains poorly understood. Here, we used multiciliated cells, targeted knockdown, and imaging to investigate function. We show that loss of causes specific depletion of outer dynein arms (ODAs) from the distal axoneme. affinity purification mass spectrometry revealed that Lrrc56 binds the ODA docking complex components, including Odad3. Consistently, knockdown also led to distal loss of Odad3. Moreover, we show that disease-associated variants in and disrupted their localization and interaction, pointing to a shared functional pathway. Our work demonstrates that is a critical regulator of distal ODAs and ODA docking complex deployment and provides new mechanistic insight into how mutations contribute to PCD.