PKD2 encodes a protein of unknown function that is mutated in 15% of autosomal dominant polycystic kidney disease (ADPKD) families. We used polyclonal antisera against PKD2 to examine the pattern of Pkd2 expression in staged mouse embryos. Staining for Pkd2 was documented as early as the 6th embryonic day (day E6) in the embryonic ectoderm and endoderm. Low-intensity staining is seen in metanephric ureteric bud at day E12.5. Byday E15.5, the adult pattern of expression is established with low level staining in proximal tubules and high level, basolateral staining in distal tubules. Pkd2 expression is first detected in the medullary collecting ducts at postnatalday 14. Outside of the kidney, Pkd2 expression is widely distributed in utero and more restricted postnatally. The greatest intensity of staining is seen in the fetal but not adult adrenal cortex and in red blood cell precursors. Expression also is seen in multiple endocrine organs, in cardiac, skeletal, and smooth muscle, and in multiple mesenchymal tissues. The diffuse distribution and early expression of Pkd2 suggest a fundamental developmental role. The persistent strong expression in adult kidney is consistent with a more organ-specific function in the maintenance of the mature metanephric tubule.