B-Raf proto-oncogene, serine/threonine kinase (BRAF)-mutated and morphologically spitzoid tumors (BAMS) are a recently described subset of melanocytic neoplasms characterized by spitzoid histomorphology in the presence of canonical BRAF mutations, most commonly BRAF V600E (valine-to-glutamic acid substitution at codon 600). Here, BRAF is linked to arhinia, choanal atresia, and microphthalmia.