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MicroRNAs (miRNAs) are small non-coding RNAs that regulate gene expression post-transcriptionally by fine-tuning mRNA levels and translation during development and in adult tissues. miRNAs are transcribed as parts of longer precursors that undergo multiple processing steps before the mature miRNAs reach their target mRNAs in the cytoplasm. In addition to Drosha/DGCR8 and Dicer that are the essential components of the miRNA processing pathway, a range of other RNA binding proteins have recently been implicated in miRNA biogenesis. Among these, several well-known splicing factors have emerged as regulators of distinct miRNAs. In this review, we examine the mechanisms by which splicing factors regulate miRNA biogenesis. As both splicing factors and miRNAs play central roles in human disease biology we discuss implications of the links between splicing factors and miRNAs in human disease.