BACKGROUND: The early diagnosis of biliary atresia (BA) is associated with a better outcome after portoenterostomy. However, very early liver biopsy findings may appear atypical for BA and delay diagnosis. Repeat biopsy histology may change rapidly to show more typical features. METHODS: Between 1997 and 2018, 6 babies with jaundice had more than one biopsy to establish diagnosis. Clinical and histologic data were collected. chi-Square was used for analysis (p < 0.05 significant). RESULTS: Five patients had two biopsies, and one had three. Median age at first, second, and third biopsy was 40 (13-57), 68.5 (35-78), and 133 days, respectively. Biopsy readings showed no portal edema initially (0/6), but in all 6 on repeat biopsy (p = 0.001). Bile duct proliferation was seen in 6/6 final biopsies, but in only 1/6 initially (p = 0.003). All patients underwent a portoenterostomy (median age 75 days (43-113)). Median delay between initial biopsy and Kasai was 29 days (14-67). Transplant free survival (n = 5 patients) ranged from 184 to 716 days (median 309 days). One patient died before being transplanted. CONCLUSION: Early biopsies may not display characteristic findings of BA, but these can appear quickly on subsequent evaluation. The interval needed to repeat a biopsy may have an adverse effect on bile drainage. LEVEL OF EVIDENCE: IV.